EDITORIAL ESPANOL:

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Hola amigos de la RED,, DERMAGIC una vez mas con ustedes, el tema: EL LUPUS ERITEMATOSO SISTÉMICO, temida enfermedad y TODO un reto para tratar. Sigo en esta onda de enfermedades conectivas con motivo de la Reunión del 22 de Mayo en Valencia cuyo tema central serán estas enfermedades,, Encontré 90 interesantes referencias al respecto que enviaré en 2 correos para no hacerlo tan pesado. 

Dr. Carlos Fachin (Valencia, Venezuela) Bienvenido a Dermagic...

Saludos,,,

Dr. José Lapenta R.,,,

 EDITORIAL ENGLISH:

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Hello friends of the NET, DERMAGIC once again with you. The topic: SYSTEMIC LUPUS ERYTHEMATOSUS, feared illness and an entire challenge to try. I continue in this wave of connective tissue diseases with reason of the Meeting of May 22 in Valencia (Venezuela) whose central topic will be these illnesses, I Found 90 interesting references in this respect, that I will send in 2 mail for not making it so heavy.  

Dr. Carlos Fachin (Valencia, Venezuela) welcome to Dermagic...

Greetings,,,

Dr. José Lapenta R. 

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DERMAGIC/EXPRESS(53)

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LUPUS ERITEMATOSO SISTÉMICO /SYSTEMIC LUPUS ERYTHEMATOSUS

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A.) 2019 European League Against Rheumatism/­American College of Rheumatology Classification Criteria for Systemic Lupus Erythematosus.

B.) 2019 European League Against Rheumatism/­American College of Rheumatology Classification Criteria for Systemic Lupus Erythematosus.

C.) Performance of the 2019 EULAR/­ACR Classification Criteria for Systemic Lupus Erythematosus in Early Disease, Across Sexes and Ethnicities.

D.) In an Early SLE Cohort the ACR-1997, SLICC-2012 and EULAR/­ACR-2019 Criteria Classify Non-Overlapping Groups of Patients: Use of All Three Criteria Ensures Optimal Capture for Clinical Studies While Their Modification Earlier Classification and Treatment.

E.) Diagnostic Accuracy of the European League Against Rheumatism/­American College of Rheumatology-2019 Versus the Systemic Lupus International Collaborating Clinics-2012 Versus the ACR-1997 Classification Criteria in Adult Systemic Lupus Erythematosus: A Systematic Review and Meta-Analysis.

1.) [A case of lupus myocarditis and nephritis with transient foramen

jugular syndrome]

2.) [Two cases with SLE and MCTD developed after a long period of chronic

arthritis that was initially diagnosed as JRA]

3.) An increased prevalence of Epstein-Barr virus infection in young

patients suggests a possible etiology for systemic lupus erythematosus.

4.) [Newer approach of screening test for antinuclear antibodies: an

enzyme-linked immunosorbent assay detecting antinuclear antibodies

characteristic of connective tissue diseases]

5.) Development of systemic lupus erythematosus in a rheumatoid arthritis

patient with anti-ribosomal P protein antibody.

6.) Elevated soluble fas production in SLE correlates with HLA status not with disease activity.

7.) Lack of NK cells in lupus patients with renal involvement.

8.) Systemic lupus erythematosus in India.

9.) Systemic lupus erythematosus (SLE) lymphadenopathy presenting with

histopathologic features of Castleman' disease: a clinicopathologic study

of five cases.

10.) [A case of systemic lupus erythematosus associated with minimal change

nephrotic syndrome]

11.) T cell receptor clonotypes in skin lesions from patients with systemic

lupus erythematosus.

12.) Circulating plasma levels of nucleosomes in patients with systemic

lupus erythematosus: correlation with serum antinucleosome antibody titers

and absence of clear association with disease activity.

13.) Autoantibodies to human recombinant erythropoietin in patients with

systemic lupus erythematosus: correlation with anemia.

14.) A promoter polymorphism of tumor necrosis factor alpha associated with

systemic lupus erythematosus in African-Americans.

15.) Histone-specific Th0 and Th1 clones derived from systemic lupus

erythematosus patients induce double-stranded DNA antibody production.

16.) Hepatitis C virus antibodies in systemic lupus erythematosus.

17.) Cutaneous lupus mucinosis: a review of our cases and the possible

pathogenesis.

18.) The immunofluorescent profile of dermatomyositis: a comparative study

with lupus erythematosus.

19.) [A man with systemic lupus erythematosus presenting with spastic

paraplegia]

20.) Cyclosporine and therapeutic plasma exchange in treatment of

progressive autoimmune diseases.

21.) [The incidence of the antiphospholipid syndrome in the clinical and

22.) Emotional and physical intimacy in coping with lupus: women's dilemmas

of disclosure and approach.

23.) [A study on interval of intravenous cyclophosphamide pulse in the

treatment of severe systemic lupus erythematosus]

24.) Autonomic nervous dysfunction in systemic lupus erythematosus (SLE) and

rheumatoid arthritis (RA): possible pathogenic role of autoantibodies to

autonomic nervous structures.

25.) [Cyclophosphamide therapy in systemic lupus erythematosus]

26.) Pulmonary involvement in systemic lupus erythematosus.

27.) Renal vein thrombosis in Chinese patients with systemic lupus

erythematosus.

28.) Usefulness of antinuclear antibody testing to screen for rheumatic

diseases.

29.) [Human parvovirus B19 infection mimicking systemic lupus erythematosus:

case report]

30.) SLE and Sjogren's syndrome associated with unilateral moyamoya vessels in cerebral arteries.

31.) SLE with death from acute massive pulmonary hemorrhage caused by

disseminated strongyloidiasis.

32.) A fatal case of severe SLE complicated by invasive aspergillosis.

33.) Methotrexate use in miscellaneous inflammatory diseases.

34.) Renal vascular lesions in lupus nephritis.

35.) Antibodies to C1q in systemic lupus erythematosus: characteristics and

relation to Fc gamma RIIA alleles.

36.) Heredity and systemic lupus erythematosus: dissecting a complex genetic

37.) Massive uncomplicated vascular immune complex deposits in the kidney of a patient with systemic lupus erythematosus.

38.) [Oral manifestations in patients with systemic lupus erythematosus]

39.) Vasculitis and bacteraemia with Yersinia enterocolitica in late-onset

systemic lupus erythematosus.

40.) Neoral--new cyclosporin for old?

41.) Successful therapy with danazol in refractory autoimmune

thrombocytopenia associated with rheumatic diseases.

42.) Predisposing factors in sulphasalazine-induced systemic lupus

erythematosus.

43.) Clinical features of lupus myositis versus idiopathic myositis: a

review of 30 cases.

44.) Serological characteristics of systemic lupus erythematosus from a

hospital-based rheumatology clinic in Kuwait.

45.) Elevated anticardiolipin antibodies in autoimmune haemolytic anaemia

irrespective of underlying systemic lupus erythematosus.

46.) A young woman with SLE: diagnostic and therapeutic challenges.

47.) Binding characteristics of SLE anti-DNA autoantibodies to modified DNA

analogues.

48.) Circulating interleukin-6 type cytokines in patients with systemic

lupus erythematosus.

49.) [Osteonecrosis in systemic lupus erythematosus. Report of 3 cases]

50.) Association of an insertion polymorphism of angiotensin-converting enzyme gene with the activity of systemic lupus erythematosus. 

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1.) [A case of lupus myocarditis and nephritis with transient foramen

jugular syndrome]

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AU: Kohro-Kawata-J; Nakamura-H; Yamamoto-T; Fukuta-S; Matsuzaki-M

SO: Ryumachi. 1997 Oct; 37(5): 709-13

AB: A 46-year-old man was admitted to our clinic because of acute heart

failure. Six years before admission he was pointed out cardiomegary and

hematuria. One year later, he was diagnosed as having jugular foramen

syndrome. On admission, he had a fever and dyspnea. Pansystolic blowing

murmur was audible at the apex. The chest ratio on his chest X-ray was

52.5%. An electrocardiogram showed left ventricular hypertrophy. An

echocardiogram showed marked dilatation and severe dysfunction of left

ventricle. Radionuclide scanning with technetium 99 m pyrophosphate

identified inflammatory change in the apex. Myocardial biopsy showed

fibrotic degeneration and IgG deposits in myocardium. Blood examination

showed anemia, lymphopenia. positive anti-nuclear antibody (1000 times,

shaggy pattern), positive anti ds-DNA antibody and hypocomplementemia.

Furthermore, proteinuria was pointed out. Renal biopsy showed focal

segmental glomerulonephritis with active necrotizing lesion (type III

nephritis). Lupus myocarditis and nephritis was diagnosed. After

prednisolone (80 mg/day) was administered. left ventricular function and

hypocomplementemia improved. The ACE inhibitor was also used for

proteinuria. In spite of a little amount of blood transfusion, he showed

hepatic hemosiderosis. We suspect that the cause of hemosiderosis was

related chronic inflammation of active lupus. It was treated with

Erythropoietin.

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2.) [Two cases with SLE and MCTD developed after a long period of chronic

arthritis that was initially diagnosed as JRA]

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AU: Takei-S; Maeno-N; Shigemori-M; Nakae-Y; Mori-H; Nerome-Y; Imanaka-H;

Hokonohara-M; Miyata-K

SO: Ryumachi. 1997 Oct; 37(5): 702-8

AB: In order to discuss the diversity of clinical features and the

difficulty in diagnosis of children with juvenile rheumatoid arthritis

(JRA), we present two cases who have documented the development of systemic

lupus erythematosus (SLE) and mixed connective tissue disease (MCTD) after

a long period of disease characterized only by arthritis that was initially

diagnosed as JRA. The first case was a girl diagnosed for her arthritic

joints as polyarticular JRA at 15 years of age. At onset, she had Raynaud

phenomenon but autoantibodies such as anti-nuclear antibody (ANA), anti-DNA

antibody, and rheumatoid factor were negative. Five years after onset, she

became ANA positive and 3 years later she became pregnant. During her

pregnancy, she became positive for anti-DNA antibody without any signs of

nephritis. One month after the delivery, however, she developed butterfly

rash, carditis, nephritis, and was diagnosed as SLE. No destructive changes

were observed in her joints though arthritis continued for 8 years form

onset to pregnancy. The second case was a 3 years old girl who was

diagnosed as polyarticular JRA. Treatment by aspirin induced complate

remission after one year from the onset. However, 10 years after that

remission, she developed Raynaud phenomenon and arthralgia in her knees and

hip joints. Her laboratory findings showed hypergammaglobulinemia, positive

ANA, positive anti-DNA antibody, positive anti-RNP antibody. She was

eventually diagnosed as MCTD when she was found to have polymyositis by EMG

and serum CK. In the present paper, two cases imply the difficulty in

diagnosing JRA and diversity of rheumatic diseases such as JRA, SLE and

MCTD. Closer and longer period of observation is essential for the JRA

patients with nondestructive arthritis.

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3.) An increased prevalence of Epstein-Barr virus infection in young

patients suggests a possible etiology for systemic lupus erythematosus.

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AU: James-JA; Kaufman-KM; Farris-AD; Taylor-Albert-E; Lehman-TJ; Harley-JB

SO: J-Clin-Invest. 1997 Dec 15; 100(12): 3019-26

AB: An unknown environmental agent has been suspected to induce systemic

lupus erythematosus (lupus) in man. Prompted by our recent immunochemical

findings, we sought evidence for an association between Epstein-Barr virus

infection and lupus. Because the vast majority of adults have been infected

with Epstein-Barr virus, we chose to study children and young adults.

Virtually all (116 of 117, or 99%) of these young patients had

seroconverted against Epstein-Barr virus, as compared with only 70% (107 of

153) of their controls (odds ratio 49.9, 95% confidence interval 9.3-1025,

P < 0. 00000000001). The difference in the rate of Epstein-Barr virus

seroconversion could not be explained by serum IgG level or by

cross-reacting anti-Sm/nRNP autoantibodies. No similar difference was found

in the seroconversion rates against four other herpes viruses. An assay for

Epstein-Barr viral DNA in peripheral blood lymphocytes established

Epstein-Barr virus infection in the peripheral blood of all 32 of the lupus

patients tested, while only 23 of the 32 matched controls were infected

(odds ratio > 10, 95% confidence interval 2.53-infinity, P < 0.002). When

considered with other evidence supporting a relationship between

Epstein-Barr virus and lupus, these data are consistent with, but do not in

themselves establish, Epstein-Barr virus infection as an etiologic factor

in lupus.

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4.) [Newer approach of screening test for antinuclear antibodies: an

enzyme-linked immunosorbent assay detecting antinuclear antibodies

characteristic of connective tissue diseases]

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AU: Asanuma-H; Miyake-J; Miyawaki-S

SO: Nihon-Rinsho-Meneki-Gakkai-Kaishi. 1997 Oct; 20(5): 417-27

AB: An enzyme-linked immunosorbent assay (ELISA) has been developed for the

detection of antinuclear antibodies (ANAs) previously established as

diagnostic and/or prognostic marker ANAs for various connective tissue

diseases. The antigen used in ELISA is a mixture of purified recombinant or

natural antigens including single-and double-stranded DNA, RNP, Sm,

SS-A/Ro, SS-B/La, centromere, topoisomerase I and Jo-1 antigens. Thirty

hundred and fifty nine patients sera from a variety of connective tissue

diseases and 113 normal human sera (NHS) were examined. ELISA ANAs were

positive in 3.5% of NHS and 80.2% of patients sera at cut off index 11.5,

whereas indirect immunofluorescent antinuclear antibodies (FANAs) using

HEp-2 cells were positive in 9.7% of NHS and 92.5% of patients sera at

1:160 serum dilution. More than 80% of sera from systemic lupus

erythematosus, mixed connective tissue disease and primary Sjogrens disease

were ELISA ANAs positive. Mean value of ELISA ANAs was highest in sera of

patients with MCTD. ELISA ANAs were positive in 92.5% of sera with marker

ANAs for connective tissue diseases. Mean value of ELISA ANAs was higher in

sera with more than two marker ANAs than in sera with a single ANA or in

sera without marker ANAs. In contrast incidence and mean value of ELISA

ANAs were low in sera positive for anti topoisomerase I antibody or anti

Jo-1 antibody. Sensitivity, specificity and agreement (accuracy) for

connective tissue diseases with marker ANAs were as follows: ELISA ANAs (at

index 11.5): 92.5%, 88.3% and 90.9%: FANAs (at 1:160 serum dilution):

99.0%, 70.4% and 88.1%, respectively. ELISA ANAs, thus, are specific for

connective tissue diseases when compared to FANAs and previous ELISA for

the detection of total ANAs. Moreover, ELISA ANAs are able to measure

precise ANAs titers and are much less labor intensive when screening a

large number of clinical specimens.

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5.) Development of systemic lupus erythematosus in a rheumatoid arthritis

patient with anti-ribosomal P protein antibody.

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AU: Hamasaki-K; Mimura-T; Kanda-H; Morino-N; Yazaki-Y; Nojima-Y

SO: Lupus. 1997; 6(9): 734-6

AB: We describe a patient with rheumatoid arthritis (RA) whose sera

contained a high titre of an antibody targeting cytoplasmic ribosomal P

proteins (anti-P). This association preceded by 6 years the development of

serological and clinical manifestations of systemic lupus erythematosus

(SLE). The clinical significance of anti-P for the diagnosis of SLE is

discussed.

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6.) Elevated soluble fas production in SLE correlates with HLA status not with disease activity.

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AU: Rose-LM; Latchman-DS; Isenberg-DA

SO: Lupus. 1997; 6(9): 717-22

AB: Evidence from animal models of lupus suggests that disruption of

Fas-mediated apoptotic events may play a role in systemic lupus

erythematosus (SLE). The recently described secreted from of Fas (sFas)

could interfere with apoptotic events by blockading Fas/Fas ligand

interactions. We describe elevated secreted Fas protein in sera from 60

patients with SLE compared with controls but neither sFas protein nor sFas

mRNA levels correlated with disease activity. At the mRNA level there is

strong evidence that individuals with human leucocyte antigens common in

SLE patients have a genetic predisposition for increased secreted Fas

production.

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7.) Lack of NK cells in lupus patients with renal involvement.

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AU: Erkeller-Yuksel-FM; Lydyard-PM; Isenberg-DA

SO: Lupus. 1997; 6(9): 708-12

AB: We have previously shown that patients with SLE have significantly

lower percentages and absolute numbers of NK(CD3-/CD16+56) cells in their

peripheral blood compared with normals. Patients with active disease had

very low levels of NK cells and the reduction was also associated with

patients who had renal involvement. We have now performed a serial study

immunophenotyping 11 patients with SLE and renal involvement using dual

colour immunofluorescence and flow cytometry. Patients were tested every

three months on an average of three occasions. As a control, nine SLE

patients without renal involvement were immunophenotyped for similar

intervals; 11 normal controls were also tested. Major lymphocyte subsets

(T, B and NK) remained very stable during serial bleeds. However, the NK

cell populations were decreased significantly in patients with renal

involvement both as percentages (5 +/- 6 vs 9 +/- 5, P < 0.0001) and

absolute counts (75 +/- 108 vs 109 +/- 52, P < 0.001) in comparison to

non-renal patients. Analysis of disease activity using BILAG score showed

an inverse correlation between renal system activity and percentage and

absolute number of NK cells (P < 0.002 and 0.01, respectively). In this

study we have also analysed a CD8 T cell subset which we have not studied

before. We have found a significantly increased percentage of CD38+CD8+ T

cells(activated CD8 subset) in patients with SLE in comparison to normal

controls. We did not find any association with the CD38+CD8+ T cells and

disease activity as measured by BILAG or renal involvement. NK cells are

important factors in immunity against virus infections and tumour cells.

CD38+CD8+T cells are increased in viral infections. We speculate that the

lack of NK cells in SLE patients might have an association with increased

CD38 expression.

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8.) Systemic lupus erythematosus in India.

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AU: Malaviya-AN; Chandrasekaran-AN; Kumar-A; Shamar-PN

SO: Lupus. 1997; 6(9): 690-700

AB: The first case of systemic lupus erythematosus (SLE) was reported from

India in 1995 followed by two more case reports and further, a series of

eight cases, till 1969. Since the establishment of a clinical immunology

laboratory at a major teaching institution in New Delhi in 1968, SLE was

extensively studied and reported from that centre. From mid-1980 onwards

several other centres in different regions in India including Chennai (old

name Madras), Mumbai (old name Bombay), Calcutta and Hydrabad, also

published their regional experience on SLE. Based on these data, the

present report describes the clinical and laboratory characteristics of

1366 SLE patients seen in different regions of India. Arthritis, rash,

photosensitivity, seizures and psychosis were seen in comparable

proportions to other racial groups. Similarly, ANA and anti-DNA antibody

positivity was also within the range seen in other racial groups. When

compared with other series, however, alopecia, renal lupus, oral ulcers and

neurological involvement was seen in higher proportions, reaching

statistically significant figures in comparison to some racial groups. In

contrast, haematological manifestations were seen in significantly less

proportions in comparison to some of the racial groups. Serositis and

discoid lesions were also seen in lower proportions than in most of other

races. The proportion of those with anti-Sm antibodies was in between two

extremes of highest among Africans and Israelis and lowest among Chinese

and Europeans. Other manifestations were comparable to most other racial

groups. Compared to North American and European reports, significantly low

5 and 10 year survival was observed among patients from India. This could

be related to the general public health situation in the country including

less than optimal management facilities in hospitals, delay in diagnosis

due to lack of awareness of the disease, referral bias where only serious

patients reach major city hospitals, or a truly severe disease among

Indians, or a combination of these genetic, environmental and/or

sociocultural factors. The Main causes of death were irreversible renal

damage, infections and neurological involvement. Despite a comparable

prevalence of anticardiolipin antibodies (aCL) and lupus anticoagulants

(LAC), clinical antiphospholipid syndrome was significantly less common.

Genetic studies showed appreciable increase of HLA DR4 (37.5%) among

patients compared with controls (18%). Additionally the haplotype B8-DR3

was encountered frequently in the patient group.

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9.) Systemic lupus erythematosus (SLE) lymphadenopathy presenting with

histopathologic features of Castleman' disease: a clinicopathologic study

of five cases.

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AU: Kojima-M; Nakamura-S; Itoh-H; Yoshida-K; Asano-S; Yamane-N;

Komatsumoto-S; Ban-S; Joshita-T; Suchi-T

SO: Pathol-Res-Pract. 1997; 193(8): 565-71

AB: Lymph node enlargement is common in active systemic lupus erythematosus

(SLE), a disease characterized by well defined clinical criteria. Although

numerous reports have described the characteristic histology of SLE

lymphadenopathy to include necrotizing lesions and hematoxylin bodies, no

detailed description has examined the histopathologic features that are

similar to Castleman's disease (CD) in SLE patients. In this report, we

describe the clinicopathologic findings of CD-like peripheral

lymphadenopathy, which was identified in five (26%) of 19 SLE patients.

These five patients were all female with an age range of 24 to 44 years,

and four of them presented with multicentric lymphadenopathy. They also had

systemic symptoms and abnormal laboratory findings, indicating active

disease, although two patients had not fulfilled the diagnostic criteria of

SLE at the initial disease. The size of the enlarged lymph nodes seldom

exceeded 2.0 cm in diameter, and biopsies revealed histopathologic features

similar to CD, of intermediate type in three patients and hyaline vascular

type in two according to the classification of Flendrig [7].

Immunohistochemical studies demonstrated polyclonal plasma cell populations

in all five cases. Epstein-Barr virus genomes were detected in the small

lymphocytes of two of the three cases examined by in situ hybridization

studies. Recently, the histopathologic findings of CD have been associated

with a disrupted immune response, and the present data suggest that SLE

should be listed as one of the diseases showing the histopathologic

features similar to CD.

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10.) [A case of systemic lupus erythematosus associated with minimal change

nephrotic syndrome]

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AU: Horita-Y; Nazneen-A; Cheng-M; Razzaque-MS; Namie-S; Tadokoro-M;

Taura-K; Miyazaki-M; Ozono-Y; Kohno-S; Harada-T; Taguchi-T

SO: Nippon-Jinzo-Gakkai-Shi. 1997 Oct; 39(7): 759-64

AB: A case of systemic lupus erythematosus (SLE) associated with minimal

change nephrotic syndrome (MCNS) in a 25-year-old female is described. The

patient suddenly manifested butterfly rash and proteinuria was first

pointed out on March, 1994. On admission, her skin biopsy indicated SLE.

Subsequently, she developed nephrotic syndrome. Urinalysis showed heavy

proteinuria (4.1 g/day), with no other abnormalities in the urinary

sediment. Immunological examination revealed positive antinuclear antibody

at a titer of 1:80 with a speckled pattern. Anti-ssDNA and anti-SS-A

antibodies were positive, but other antibodies were negative. Serum

complement (CH50) was within the normal range (30.5 U/ml). The renal biopsy

showed no apparent cellular proliferation or increase of extracellular

matrices in glomeruli by light microscopy. Slight deposition of IgG, IgM,

C3 and C1q was focally seen in the mesangium and capillary wall by

immunofluorescence. Electron microscopic examination revealed small and

scattered dense deposits in the mesangium, subepithelium and

subendothelium, associated with diffuse fusion of the foot processes of

epithelial cells along the glomerular basement membrane. According to the

WHO classification, the histological features were compatible with those of

lupus nephritis (LN), class Ib. The patient was treated with PREDNISOLONE,

Mizorbine and Dilazep, resulting in the disappearance of proteinuria and a

normal serum level of total protein. The association of LN and MCNS is very

rare. We also investigated the relationship between the intensity of

proteinuria and histological types of 53 cases with LN examined in our

laboratory. The cases with heavy proteinuria were mostly classified as

WHO-Class IV and Class V. We report here a case of LN associated with MCNS

and also review the literatures.

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11.) T cell receptor clonotypes in skin lesions from patients with systemic

lupus erythematosus.

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AU: Kita-Y; Kuroda-K; Mimori-T; Hashimoto-T; Yamamoto-K; Saito-Y;

Iwamoto-I; Sumida-T

SO: J-Invest-Dermatol. 1998 Jan; 110(1): 41-6

AB: Systemic lupus erythematosus is an autoimmune disease characterized by

the presence of autoantibodies and by lymphocytic infiltration into lesions

at several sites such as skin, kidney, and other organs. Immunohistologic

studies have clarified that the majority of lymphocytes in the skin are

CD4+ alphabeta T cells. In the present work, to clarify the pathologic role

of T cells in the skin of systemic lupus erythematosus patients, we

analyzed T cell receptor (TCR) clonotypes of T cells infiltrating into skin

lesions. TCR Vbeta gene transcripts from T cells from discoid lesions of

the skin and peripheral blood lymphocytes of four systemic lupus

erythematosus patients were amplified by reverse transcriptase polymerase

chain reaction. Southern blot analysis of polymerase chain reaction product

demonstrated the heterogeneous TCR Vbeta repertoire of T cells in the skin

of systemic lupus erythematosus. Single-strand conformation polymorphism

analysis showed several distinct bands for smears of most TCR Vbeta genes

from T cells infiltrating the skin, whereas smears with few bands were

found for all TCR Vbeta genes from peripheral blood lymphocytes of the same

patients. The number of bands encoding each TCR Vbeta gene from the skin

was significantly higher compared with peripheral blood lymphocytes.

Sequencing analysis showed a Leucine-X-Glycine amino acid motif at position

96-98 in the CDR3 region at the frequency of 23-24% in skin-accumulated T

cells from two patients, whereas the frequency of this motif in peripheral

T cells was only 0-3%, indicating limited T cell epitopes. In conclusion, T

cells infiltrating into the skin of systemic lupus erythematosus patients

might recognize restricted T cell epitopes on autoantigens and trigger the

autoimmune reaction in skin lesions.

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12.) Circulating plasma levels of nucleosomes in patients with systemic

lupus erythematosus: correlation with serum antinucleosome antibody titers

and absence of clear association with disease activity.

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AU: Amoura-Z; Piette-JC; Chabre-H; Cacoub-P; Papo-T; Wechsler-B; Bach-JF;

Koutouzov-S

SO: Arthritis-Rheum. 1997 Dec; 40(12): 2217-25

AB: OBJECTIVE. To assess nucleosome plasma levels in patients with systemic

lupus erythematosus (SLE) and to study the correlations with serum

antinucleosome, anti-double-stranded DNA (anti-dsDNA), and antihistone

antibody activities, as well as with disease activity (by the SLE Disease

Activity Index [SLEDAI]). METHODS. In a cross-sectional study, we assessed

58 SLE patients for their plasma nucleosome levels. Plasma nucleosome

levels as well as serum antinucleosome, anti-double-stranded DNA, and

antihistone antibody activities were assessed by enzyme-linked

immunosorbent assay. SLE activity was evaluated using the SLEDAI. RESULTS.

The mean (+/-SD) plasma nucleosome concentration in SLE patients was 52 +/-

159 ng/ml (range 5-1,180), and was significantly higher than that of the

controls (16 +/- 8.8 ng/ml, range 8-52; P = 0.03). Thirteen of the 58 lupus

patients had levels over the range of normal (defined as the control mean +

3 SD, or 42 ng/ml). An inverse correlation was found between nucleosome

plasma levels and serum antinucleosome antibody activity in the entire

group of SLE patients, those with active disease, and those with inactive

disease, respectively. No correlation was found between the SLEDAI and

nucleosome plasma concentrations. CONCLUSION. Nucleosome plasma levels may

be normal or increased in SLE, and found in patients with active or

inactive SLE. Longitudinal studies are needed to further establish whether

high levels of circulating nucleosomes may predict the occurrence of an SLE

flare.

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13.) Autoantibodies to human recombinant erythropoietin in patients with

systemic lupus erythematosus: correlation with anemia.

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AU: Tzioufas-AG; Kokori-SI; Petrovas-CI; Moutsopoulos-HM

SO: Arthritis-Rheum. 1997 Dec; 40(12): 2212-6

AB: OBJECTIVE. To investigate the existence of circulating autoantibodies

to erythropoietin (EPO) in sera from patients with systemic lupus

erythematosus (SLE), and to correlate their presence with anemia and

clinical activity. METHODS. Ninety-two consecutive patients with SLE, 80

patients with rheumatoid arthritis, and 42 normal individuals were studied.

The patients with SLE were categorized into 3 groups according to

hemoglobin (Hgb) level: group A (45 patients with Hgb > 12 gm/dl), group B

(26 patients with Hgb 10.1-12 gm/dl), and group C (21 patients with Hgb <

or = 10 gm/dl). In all patients with SLE, the disease activity was

evaluated using the European Consensus Lupus Activity Measurement scale.

Antibodies to EPO were detected using an enzyme-linked immunosorbent assay

and purified recombinant human EPO as antigen. The specificity of the

method was evaluated with homologous and cross-reactive inhibition assays.

RESULTS. Antibodies to EPO were found in 15.2% of the SLE patient sera. The

distribution of these antibodies among the 3 groups of SLE patients was as

follows: 8.8% (4 of 45) from group A, 15.4% (4 of 26) from group B, and

28.6% (6 of 21) from group C. The prevalence of antibodies to EPO in

patients with severe anemia (group C) was statistically significantly

higher compared with patients without anemia (chi(2) = 4.31, P < 0.05).

Patients with antibodies to EPO had higher disease activity scores (P <

0.005) and lower levels of the C4 component of complement (P < 0.05)

compared with patients without antibodies to EPO. CONCLUSION. In this

study, the presence of antibodies to EPO in the sera of SLE patients is

demonstrated for the first time. The presence of these antibodies is

associated with severe anemia and active disease.

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14.) A promoter polymorphism of tumor necrosis factor alpha associated with

systemic lupus erythematosus in African-Americans.

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AU: Sullivan-KE; Wooten-C; Schmeckpeper-BJ; Goldman-D; Petri-MA

SO: Arthritis-Rheum. 1997 Dec; 40(12): 2207-11

AB: OBJECTIVE. The polymorphic tumor necrosis factor alpha (TNFalpha) gene

encodes a cytokine involved in inflammation, angiogenesis, and apoptosis.

One polymorphic variant is associated with increased production of

TNFalpha. This study examined the frequency of this polymorphic variant in

African-American patients with systemic lupus erythematosus (SLE) compared

with controls. METHODS. We determined the gene frequency of the polymorphic

variant of TNFalpha in an African-American SLE patient population and in a

geographically matched African-American control population. RESULTS. The

gene frequency of the TNFalpha -308A polymorphism was higher in the

African-American SLE population than in the control population. This

relationship was independent of major histocompatibility complex DR

alleles. CONCLUSION. The TNFalpha -308A polymorphism is associated with an

increased risk of SLE in African-Americans.

=====================================================================

15.) Histone-specific Th0 and Th1 clones derived from systemic lupus

erythematosus patients induce double-stranded DNA antibody production.

=====================================================================

AU: Voll-RE; Roth-EA; Girkontaite-I; Fehr-H; Herrmann-M; Lorenz-HM; Kalden-JR

SO: Arthritis-Rheum. 1997 Dec; 40(12): 2162-71

AB: OBJECTIVE. To investigate whether histone-specific T helper (Th) cells

that are able to induce anti-double-stranded DNA (anti-dsDNA) antibodies

can be isolated from patients with systemic lupus erythematosus (SLE) and

to characterize the cytokine secretion pattern of such Th clones. METHODS.

Peripheral blood mononuclear cells from SLE patients and healthy donors

were stimulated with autologous apoptotic cell material or purified

histones, expanded with interleukin-2 (IL-2), and cloned by limiting

dilution. Histone reactivity of clones was examined by histone-specific

proliferation and cytokine release. Cytokines were determined by

enzyme-linked immunosorbent assay (ELISA) and CTLL-2 bioassay. Induction of

anti-dsDNA antibodies was measured in cocultures of autologous B cells and

Th clones by ELISA. RESULTS. Numerous histone-specific T cell receptor

(TCR) alpha/beta+ Th clones were established from 2 of 3 patients with

active SLE and from 1 of 2 healthy individuals. Most Th clones secreted

IL-2, interferon-gamma (IFNgamma), and IL-4, whereas some produced

predominantly IL-2 and IFNgamma. Th clones that could stimulate the

production of anti-dsDNA antibodies were derived from SLE patients and from

a healthy individual. CONCLUSION. Th cells specific for histones may play

an important role in the pathogenesis of SLE by inducing autoantibodies to

dsDNA. Both Th1 and Th2 cytokines may be involved in the pathogenesis of

SLE. The presence of histone-specific Th cells in a healthy individual

indicates the importance of peripheral tolerance for preventing

autoimmunity to nuclear antigens.

=====================================================================

16.) Hepatitis C virus antibodies in systemic lupus erythematosus.

=====================================================================

AU: Kowdley-KV; Subler-DE; Scheffel-J; Moore-B; Smith-H

SO: J-Clin-Gastroenterol. 1997 Sep; 25(2): 437-9

AB: To determine the prevalence and significance of serum antibody to

hepatitis C virus (HCV) in patients with systemic lupus erythematosus

(SLE), we measured serum antibodies to HCV by enzyme-linked immunosorbent

(ELISA) and by Abbott MATRIX Immunoblot assays in 42 patients with SLE, a

condition associated with hypergammaglobulinemia. We used the polymerase

chain reaction (PCR) to identify patients with HCV viremia. Five of 42

(11.9%) patients were seropositive for anti-HCV by ELISA; of these only two

were positive by PCR; only one of three patients seropositive by Immunoblot

assay was also positive by PCR. Both ELISA and the Immunoblot assays may be

falsely positive for ongoing HCV infection in patients with SLE. Suspected

HCV infection should be confirmed with PCR for serum HCV RNA in these

patients.

=====================================================================

17.) Cutaneous lupus mucinosis: a review of our cases and the possible

pathogenesis.

=====================================================================

AU: Kanda-N; Tsuchida-T; Watanabe-T; Tamaki-K

SO: J-Cutan-Pathol. 1997 Oct; 24(9): 553-8

AB: Cutaneous lupus mucinosis (CLM) is a rare variant of lupus

erythematosus eruptions. Our 5 cases with CLM were reviewed. All were men

with systemic lupus erythematosus (SLE). CLM occurred as asymptomatic

cutaneous papules, nodules, or plaques on the trunk, upper and lower

extremities, and face. Histopathology of CLM mainly revealed abundant mucin

deposits among splayed collagen bundles throughout the dermis. However,

some CLM lesions showed discoid lupus erythematosus-like epidermal and

dermal changes and/or lupus profundus. Vasculitis was also revealed in the

CLM lesions of 2 cases. The pathogenesis of CLM may be closely related to

its two important features, the male preponderance and the association with

SLE. Vasculopathy may also be involved in the development of CLM.

=====================================================================

18.) The immunofluorescent profile of dermatomyositis: a comparative study

with lupus erythematosus.

=====================================================================

AU: Magro-CM; Crowson-AN

SO: J-Cutan-Pathol. 1997 Oct; 24(9): 543-52

AB: We have demonstrated a role for microvascular injury mediated by the

membrane attack complex of complement (C5b-9) in the genesis of cutaneous

lesions of dermatomyositis (DM) (1). The purpose of this study is to

revisit the immunofluorescent (IF) profile of DM, to further investigate

the role of C5b-9 in the pathogenesis of cutaneous lesions, and to see if

any features of the IF profile reliably distinguish DM from LE. Lesional

skin biopsies from 24 patients with clinical findings characteristic of DM

were received in formalin and in Michel's transport medium. Conventional

light microscopy, and IF studies with antibodies monospecific for IgG, IgA,

IgM, C3, fibrin and C5b-9 were performed. The control group comprised

biopsies from 31 patients with well-documented LE. A positive lupus band

test (LBT) correlated highly with a diagnosis of LE, with a sensitivity of

64.5% and a specificity of 95.6% (p=0.001). The LBT was most sensitive in

the setting of DLE and SLE and was least sensitive in the setting of SCLE.

The finding of vascular C5b-9 deposition correlated with a diagnosis of DM

versus LE (p=0.001) although the false positive rate was 21.4%. The false

negative rate was reduced when vascular C5b-9 was seen in the absence of

antibodies to Ro, La, or RNP. While a negative LBT correlated with a

diagnosis of DM (p=0.001), the specificity was only 64.5%. However, when it

was seen in concert with C5b-9 along the DEJ, specificity was increased to

80.6% (p=0.001). The presence of C5b-9 in vessels and along the DEJ in

concert with a negative LBT was predictive of DM (p=0.001) with a

specificity of 93.5%, sensitivity of 78.3%, a false positive rate of 10%

and a false negative rate of 14.7%. The combination of a negative LBT,

vascular C5b-9 deposition and negative serology for Ro, La, and RNP was a

predictor of DM versus LE with a sensitivity of 90.5%, a specificity of

96.8%, a false positive rate of 5% and a false negative rate of 6.2%

(p=0.001). The IF profile of DM in lesional skin comprises a negative LBT,

deposition of C5b-9 within vessels and along the DEJ, and variable

keratinocyte decoration for IgG and C5b-9. The most statistically powerful

predictor of DM is the combination of a negative LBT with vascular C5b-9

deposition and negative serology for antibodies to Ro, La, Sm, and RNP.

Demonstration of a negative LBT in all but 1 case of DM suggests that the

DEJ is not a primary site for antigen-antibody interaction. We postulate

that the aforementioned IF findings reflect humorally mediated injury of

endothelium and keratinocytes, effected by C5b-9.

=====================================================================

19.) [A man with systemic lupus erythematosus presenting with spastic

paraplegia]

=====================================================================

AU: Kashihara-K; Shiro-Y; Shohmori-T; Nomura-A; Hara-H

SO: No-To-Shinkei. 1997 Oct; 49(10): 915-8

AB: We report a 38-year-old man systemic lupus erythematosus who presented

with an acute onset of paraplegia and urinary retention. The man had a

12-year history of nodular cutaneous mucinosis and arthralgia. In 1994, he

was admitted to our hospital with a sudden onset of weakness and numbness

of the right leg followed by an emergence of similar symptoms in the left

leg. His elder sister had died at 16 years of age after suffering from

systemic lupus erythematosus for 6 years. On examination, the patient had

skin rash on his chest, back, head, forehead, and extremities. The

neurological examination revealed that his tongue deviated to the right on

protrusion. The muscle power was reduced to 2-3/5 in the right leg and to

4/5 in the left leg. The sensory disturbance was noted in the lower

extremities with predominant involvement of the right leg. Reflexes were

increased in the right biceps, triceps, both patellas, and Achilles

tendons. Babinski sign was noted bilaterally. Urinary retention and

constipation were also noted. The results of the blood cell count and

hepatic and renal function tests were normal. Serum levels of C-reactive

protein and complements (C3, C4, CH50) were also normal. Serological

examinations showed increased anti-DNA antibody (14 U/ml, [normal, < 6]).

Antinuclear antibody was positive at a titer of 1:1380. CSF study showed an

increased protein concentration of 83 mg/dl and an IgG level of 14 mg/dl

with a normal number of cells. MR images revealed a T1-low, T2-high signal

lesion at the upper part of the left ventral medulla. MR images of the

brain and spinal cord were normal. The patient was diagnosed as having SLE.

High-dose intravenous methylprednisolone (1 g/day) pulse treatment that was

started 25 days after the onset of neurological symptoms, produced partial

relief. Our case presented with paraplegia with a focal lesion in the left

upper ventral part of the medulla on MR images. The incidence of male SLE

is low, and paraplegia is a rare complication of SLE. Thus, the medullary

lesion in SLE observed in our case appears to be rare. SLE should be

considered as a cause of acute onset paraplegia or myelopathy.

=====================================================================

20.) Cyclosporine and therapeutic plasma exchange in treatment of

progressive autoimmune diseases.

=====================================================================

AU: Schiel-R; Bambauer-R; Latza-R; Klinkmann-J

SO: Artif-Organs. 1997 Sep; 21(9): 983-8

AB: Despite treatment with intensive immunosuppressive drug regimens, the

prognosis of patients suffering from severe progressive autoimmune diseases

like systemic lupus erythematosus (SLE), nephrotic syndrome (NS), and

Behcet's disease is poor. Side effects (infections and malignant tumors)

often occur. In the present trial, 35 patients suffering from autoimmune

diseases (SLE, n = 21; NS, n = 10; and Behcet's disease, n = 4) were

treated for 3.7 +/- 2.0 years with 2.5 +/- 0.6 mg cyclosporine/kg body

weight/day in addition to corticosteroids alone or in combination with

azathioprine and/or cyclophosphamide. In active stages of the diseases with

extremely high concentrations of anti-ds-DNA-antibodies, antinuclear

antibodies, circulating immunocomplexes, and reduced complement

concentrations, therapeutic plasma exchange (TPE) has been applied.

Compared with previous treatment modalities, significantly (p < 0.05) more

effective and rapid reductions of the antibodies were reached. Clinical

disorders improved within 1-6 weeks. All patients reported increased

performance and a better quality of life. After 1-12 months, the previously

required doses of immunosuppressive drugs and the frequency of TPE could be

reduced by 40-100%. After 13.4 +/- 11.8 months in 17 of 35 patients (8 with

SLE, 5 with NS, 4 with Behcet's disease), cyclosporine was established as

the monotherapy. No severe side effects were registered. In treating active

stages of severe progressive autoimmune diseases and forms with persistent

high antibody levels, the addition of TPE to conventional therapy was very

effective, as observed in both clinical and laboratory parameters.

=====================================================================

21.) [The incidence of the antiphospholipid syndrome in the clinical and

=====================================================================

biological manifestations of systemic lupus erythematosus]

AU: Sanchez-Rodriguez-A; Martin-Oterino-JA; Fidalgo-Fernandez-MA;

Araoz-Sanchez-P; Alonso-Garcia-P; Fernandez-Navarro-P; Chimpen-Ruiz-V;

Portugal-Alvarez-J

SO: Rev-Clin-Esp. 1997 Oct; 197(10): 669-74

AB: In the present investigation a study was made on the incidence of

antiphospholipid syndrome (APS) on clinical and biological manifestations

in a series or 32 patients (28 females and 4 males with a mean age of 25

years) diagnosed of SLE (ARA criteria) and APS (Harris criteria) compared

with a group of 25 patients (19 females and 6 males with a mean age of 38

years) diagnosed of SLE without APS. This entails a selection from 124

patients diagnosed of SLE, and an incidence of 25.8% and 9.7% for ACA and

LA, respectively. After a clinical protocol was filled, a complete

immunological profile was obtained, with lymphocyte subsets, IL-2 receptor,

coagulation study, isotype determination for anticardiolipin antibody

(ACA), lupus anticoagulant (LA), serology for syphilis and imaging

diagnostic techniques. Comparative results, with an statistic assessment,

are shown in tables. It is concluded that SLE + APS population can be

considered as definite for a peculiar SLE subtype.

=====================================================================

22.) Emotional and physical intimacy in coping with lupus: women's dilemmas

of disclosure and approach.

=====================================================================

AU: Druley-JA; Stephens-MA; Coyne-JC

SO: Health-Psychol. 1997 Nov; 16(6): 506-14

AB: This study examined whether self-rated physical and emotional intimacy

of 74 women with their heterosexual partner, during an illness episode of

lupus, was related to their affect and relationship satisfaction. It was

predicted that greater intimacy would be related to better psychosocial

adjustment. Women who engaged in physically intimate behavior with their

partner more often reported greater relationship satisfaction. Women who

frequently avoided or who were often the initiators of physical intimacy,

however, reported greater negative affect. Concerning emotional intimacy,

women who disclosed more information about illness symptoms and women who

concealed more information about their symptoms and feelings experienced

the highest levels of negative affect. Results identify dilemmas that women

with recurrent illness may face when trying to maintain intimacy during

illness periods.

=====================================================================

23.) [A study on interval of intravenous cyclophosphamide pulse in the

treatment of severe systemic lupus erythematosus]

=====================================================================

AU: Yang-X; Yin-P; Gao-X

SO: Chung-Hua-Nei-Ko-Tsa-Chih. 1996 Apr; 35(4): 257-60

AB: The aim of this study was to compare the clinical efficacy and adverse

effects of intravenous cyclophosphamide pulse therapy between 2-week and

4-week intervals in the treatment of severe systemic lupus erythematosus.

52 patients were involved in the study and were equally divided into two

groups. The pulse intervals were 2 weeks for group I and 4 weeks for group

II. Each pulse dose of cyclophosphamide was 15 mg/kg. Every patient took

prednisone 1 mg/kg daily for first 8 weeks and then tapered. Most of

disease indices recovered quicker in group I than in group II. The group

with short interval was better than that of long one on total efficacy and

remission rate. The cyclophosphamide cumulative doses were not different

between two groups as disease activity index dropped 50%. The pulse

interval had to be prolonged from 2 weeks to 4 weeks to six patients in

group I (23.1%) because of leukopenia, and be shortened from 4 weeks to 2

weeks to five cases in group II (19.2%) because of inefficacy. There was no

statistical difference in adverse effects between two groups. Intravenous

cyclophosphamide pulse therapy with two-weeks interval is advantageous to

recovery of severe systemic lupus erythematosus provided blood test prior

to each pulse. We suggest that pulse interval should be individualized.

=====================================================================

24.) Autonomic nervous dysfunction in systemic lupus erythematosus (SLE) and

rheumatoid arthritis (RA): possible pathogenic role of autoantibodies to

autonomic nervous structures.

=====================================================================

AU: Maule-S; Quadri-R; Mirante-D; Pellerito-RA; Marucco-E; Marinone-C;

Vergani-D; Chiandussi-L; Zanone-MM

SO: Clin-Exp-Immunol. 1997 Dec; 110(3): 423-7

AB: Autonomic nervous dysfunction has been previously reported in SLE, RA

and systemic sclerosis, but the pathogenesis of such a complication is

poorly understood. In the present study, four standard cardiovascular

autonomic function tests were performed in 34 female patients with

connective tissue diseases and in 25 healthy control subjects, and results

expressed as cardiovascular (CV) test scores. Moreover, in each subject the

presence of circulating complement-fixing autoantibodies directed against

sympathetic and parasympathetic nervous structures, represented by superior

cervical ganglia and vagus nerve, respectively, was simultaneously assessed

by an indirect immunofluorescent complement-fixation technique, using

rabbit tissue as substrate. None of the patients reported autonomic

symptoms. However, an abnormal CV test score (> or = 5) was detected in 15%

of the patients and in none of the healthy control subjects, approaching

statistical significance (P = 0.07). No correlation was found between CV

test results and disease duration, type of therapy or presence of

conventional autoantibodies. One or two autoantibodies to autonomic nervous

structures were detected in six patients (18%) and not in the control

subjects (P < 0.05). Values of deep breathing test were significantly lower

in autoantibody-positive patients compared with those amongst the control

subjects (P < 0.05), and an abnormal CV test score was significantly

associated with the presence of autoantibodies to autonomic nervous

structures (P < 0.05). In conclusion, we confirm that autonomic nervous

function can be impaired in patients with connective tissue diseases, and

suggest that autoantibodies directed against autonomic nervous system

structures may play a role in the pathogenesis of the autonomic dysfunction.

=====================================================================

25.) [Cyclophosphamide therapy in systemic lupus erythematosus]

=====================================================================

AU: Backhaus-M; Hiepe-F

SO: Z-Rheumatol. 1997 Jul-Aug; 56(4): 178-89

AB: Cyclophosphamide is a potent immunosuppressive drug which is widely

used to treat renal and central nervous system manifestation of Systemic

Lupus Erythematosus (SLE). It is employed especially to prevent renal

failure in lupus nephritis. Within the last decade intravenous

cyclophosphamide bolus therapy has become the standard therapy in severe

SLE due to its tendency towards a higher efficacy and fewer side effects as

compared to oral application. Studies on the slightly more cost-effective

oral cyclophosphamide bolus therapy have recently been published, however,

there are no data on long-term results yet. Here, we review the effects and

side effects of cyclophosphamide as well as recent clinical studies on

cyclophosphamide bolus therapy in SLE. Because of the possible side

effects, especially the high risk of malignancies, which sharply increases

at a cumulative dose of approximately 60 g, cyclophosphamide should be used

cautiously. Cyclophosphamide bolus therapy should only be performed in

hospitals with special experience.

=====================================================================

26.) Pulmonary involvement in systemic lupus erythematosus.

=====================================================================

AU: Fishback-N; Koss-MN

SO: Curr-Opin-Pulm-Med. 1995 Sep; 1(5): 368-75

AB: Pulmonary disorders in systemic lupus erythematosus involve a variety

of clinical presentations and pathologic patterns, which can be difficult

to diagnose due to systemic dysfunction, infection, or complications of

therapy. The causes of dyspnea in systemic lupus erythematosus are

multifactorial, and the clinical manifestations of lung disease widely

vary. Biopsy is frequently relied on to evaluate and diagnose pulmonary

disease in systemic lupus erythematosus. The patient who has systemic lupus

erythematosus-associated lung disease is effectively treated with various

immunosuppressive drugs, in conjunction with careful evaluation of the

patient's systemic involvement, drug-induced complications, and the

ever-present threat of infection.

=====================================================================

27.) Renal vein thrombosis in Chinese patients with systemic lupus

erythematosus.

=====================================================================

AU: Lai-NS; Lan-JL

SO: Ann-Rheum-Dis. 1997 Sep; 56(9): 562-4

AB: OBJECTIVES: To evaluate the risk factors associated with renal vein

thrombosis (RVT) in Chinese patients with systemic lupus erythematosus

(SLE). METHODS: Data on clinical symptoms, renal biopsy, antiphospholipid

antibody syndrome profile, and serological examinations of lupus features

were examined retrospectively in six patients with RVT confirmed by

angiography from a total of 625 patients with SLE over a 14 year period

(1982-1996). RESULTS: The lupus patients with RVT did not have acute

symptoms of severe flank pain, haematuria, and oligouria. In contrast, most

patients were suspected to have RVT because of peripheral oedema and

worsening proteinuria. Roentgenological examinations (including renal

sonography, renal computer tomography, or renal Doppler, or all three) were

positive only in some patients. Positive antiphospholipid antibody profiles

were found in four of six lupus patients. By renal biopsy, only two samples

were confirmed as World Health Organisation (WHO) class V lupus membranous

glomerulonephritis. The others were class IV in three patients, and class

III in the remaining one. No RVT was found in lupus patients without

nephrotic syndrome. Peripheral thrombophlebitis was, however, noted in only

one patient. CONCLUSION: Nephrotic syndrome could be a distinct risk factor

in the development of RVT in Chinese SLE patients, in contrast with that

reported in white populations in whom the peripheral thrombotic events were

recognised as a determining factor.

=====================================================================

28.) Usefulness of antinuclear antibody testing to screen for rheumatic

diseases.

=====================================================================

AU: Malleson-PN; Sailer-M; Mackinnon-MJ

SO: Arch-Dis-Child. 1997 Oct; 77(4): 299-304

AB: OBJECTIVE: To assess the usefulness of the indirect immunofluorescence

antinuclear antibody test (FANA) using human laryngeal epithelial carcinoma

cells as nuclear substrate, to screen for childhood rheumatic diseases.

STUDY DESIGN: A review of all FANA tests performed on children at British

Columbia's Children's Hospital between 7 March 1991 and 31 July 1995.

RESULTS: FANA tests were positive at titres of 1:20 or greater in 41% of

all subjects tested, and in 65% of all subjects in whom the diagnosis was

obtained. FANA positivity occurred in 67% of those with a rheumatic

disease, compared with 64% of those with a non-rheumatic disease (p = 0.4).

More girls had high titre FANA positivity than boys independent of whether

or not they had a rheumatic disease (p = 0.05). At a screening serum

dilution of 1:40 a positive test has a sensitivity of only 0.63, and a

positive predictive value of only 0.33 for any rheumatic disease. For

systemic lupus erythematosus (SLE), mixed connective tissue disease (MCTD),

or overlap syndrome at a screening dilution of 1:40 the test has a very

high sensitivity of 0.98, but a very low positive predictive value of only

0.10, the test having slightly better characteristics for boys than girls.

CONCLUSION: Although a negative FANA test makes a diagnosis of SLE or MCTD

extremely unlikely, a positive test even at moderately high titres of 1:160

or higher is found so frequently in children without a rheumatic disease

that a positive result has little or no diagnostic value. It is suggested

that a screening serum dilution of 1:160 or 1:320 would increase the

usefulness of the test, by decreasing false positive tests, without

significantly increasing false negative tests for SLE or MCTD, and would

have the potential for considerable cost savings.

=====================================================================

29.) [Human parvovirus B19 infection mimicking systemic lupus erythematosus:

case report]

=====================================================================

AU: Banno-S; Matsumoto-Y; Sugiura-Y; Ueda-R

SO: Ryumachi. 1997 Aug; 37(4): 581-6

AB: Human parvovirus B19 (HPV-B19) has been known as the etiologic agents

of erythema infectiosum in normal childhood, and chronic anemia and

thrombocytopenia in immuno-compromised patients. Recently, this virus has

been reported as the association with rheumatic manifestation such as

rheumatoid arthritis and systemic lupus erythematosus (SLE). We described

here a patient whose HPV-B19 infection was mimiking atypical symptoms of

SLE at diagnosis, and was persistent because of immuno-suppressive therapy

for SLE. A 34-year-old female was admitted to our hospital on 22 June 1995,

presenting fever episode and cervical lymph node swelling. Before eighteen

months, she was received methyl-predonisolone pulse therapy and plasma

exchange by fresh frozen plasma for the treatment of Stevens-Johnson

syndrome, and after several weeks these therapy she was suffered from viral

infection with lymphadenopathies with a transient appearance of atypical

lymphocytes in her peripheral blood smear. On laboratory examination at the

present admission, her peripheral blood showed anemia, thrombocytopenia

with atypical lymphocytes. Throughout her hospitalization, anti-nuclear

antibody (ANA) suspected SLE including anti-DNA and anti-Sm antibody were

all negative except of transient week positive ANA screening test. Her

physical condition presented poor clinical course with fever elevation,

increased ascites and renal dysfunction showing the elevation of CRP and

circulating immune-complex (Clq binding method). Her serum was positive for

IgM and IgG antibody against VP-1 and VP-2 antigen of HPV-B19 by ELISA in

April 1996. And then, HPV-B19 DNA by polymerase chain reaction (PCR) was

positive in bone marrow sample in March 1996, and also positive in spleen

necropsy at death. We confirmed persistent chronic HPV-B19 infection by

measurement of HPV-B19 IgM and IgG antibody by ELISA and HPV-B19 DNA by

PCR. The plasmapheresis and administration of intravenous immunoglobulin

showed the possible efficacy for her symptom throughout this clinical

course. Moreover, bone marrow smear showed the finding of virus-associated

hemophagocytic syndrome, and finally, she was died of cervical hemorrhage

accompanied with disseminated intravascular coagulation syndrome on July

1996. HPV-B19 infection can present an atypical clinical picture that is

highly suggestive of SLE. We suggest that the therapy of steroids and

immuno-suppressive agents should be cautious, because these may potentially

cause persistent chronic HPV-B19 infection and induced life-threatening

clinical course.

=====================================================================

30.) SLE and Sjogren's syndrome associated with unilateral moyamoya vessels

in cerebral arteries.

=====================================================================

AU: Matsuki-Y; Kawakami-M; Ishizuka-T; Kawaguchi-Y; Hidaka-T; Suzuki-K;

Nakamura-H

SO: Scand-J-Rheumatol. 1997; 26(5): 392-4

AB: Moyamoya disease is a rare clinical entity, diagnosed by cerebral

angiography and characterized by occlusion of the internal carotid artery

system and the development of collateral arteries. A 30-year-old woman with

systemic lupus erythematosus and Sjogren's syndrome recurrently presented

transient right homonymous hemianopsia. Cerebral angiography showed

occlusion of the left posterior cerebral artery associated with the

development of collateral circulation ("moyamoya vessels"). In a young

adult, as in this case, the unilaterality of the lesion and the

presentation of transient ischemic attacks rather than subarachnoid

hemorrhage are rare features for Moyamoya disease. Antiphospholipid

syndrome was absent.

=====================================================================

31.) SLE with death from acute massive pulmonary hemorrhage caused by

disseminated strongyloidiasis.

=====================================================================

AU: Setoyama-M; Fukumaru-S; Takasaki-T; Yoshida-H; Kanzaki-T

SO: Scand-J-Rheumatol. 1997; 26(5): 389-91

AB: We describe a case of disseminated strongyloidiasis involving a female

patient with active systemic erythematosus (SLE). The cause of death was

massive pulmonary hemorrhage induced by a filariform larvae infection. This

was initially diagnosed during examination of the bronchoalveolar lavage

fluid just 2 days before her death. The case indicated that

immunosuppressed individuals would be paid attention to possible parasitic

infection prior to starting therapy even in non-endemic areas as well as

other microorganisms.

=====================================================================

32.) A fatal case of severe SLE complicated by invasive aspergillosis.

=====================================================================

AU: Zuber-M; Daus-H; Koch-B; Pfreundschuh-M

SO: Rheumatol-Int. 1997; 17(3): 127-30

AB: We report on the case of a 25-year-old female with severe systemic

lupus erythematosus (SLE) who presented with pancytopenia, fever,

arthralgia and abdominal pain. After antibiotic treatment, the patient was

afebrile for 3 days before her temperature rose again. Dyspnoea and cough

pointed towards pneumonia which was confirmed by X-ray. Different

antibiotics and the antimycotic agent fluconazol were given. The lupus

flare was treated with high-dose prednisolone. After a couple of days, the

dyspnoea increased and mechanical ventilation became necessary.

Bronchoscopy and transbronchial biopsy revealed the diagnosis of invasive

aspergilloses. Despite of an immediate treatment with amphotericin B, the

patient died because of respiratory insufficiency. The literature on

aspergillosis in SLE is reviewed and prophylactic, diagnostic and

therapeutic options are discussed for this infectious complication which

has an 80% mortality in patients with SLE.

=====================================================================

33.) Methotrexate use in miscellaneous inflammatory diseases.

=====================================================================

AU: Wilke-WS

SO: Rheum-Dis-Clin-North-Am. 1997 Nov; 23(4): 855-82

AB: Methotrexate has proven to be a safe, effective, long-term therapy for

rheumatoid arthritis. Its property as a corticosteroid-sparing drug in

rheumatoid arthritis has been recognized and its potential has been

explored in other inflammatory and autoimmune diseases. This article

describes and analyzes the use of methotrexate for a wide variety of

diseases, some of which are not the usual province of rheumatologists, to

provide some guidance concerning its role for treatment. Methotrexate

therapy seems promising for systemic lupus erythematosus, inflammatory

myopathy, inflammatory eye disease, inflammatory bowel disease, and some

manifestations of sarcoidosis. Its role in other diseases is not as well

defined.

=====================================================================

34.) Renal vascular lesions in lupus nephritis.

=====================================================================

AU: Descombes-E; Droz-D; Drouet-L; Grunfeld-JP; Lesavre-P

SO: Medicine-Baltimore. 1997 Sep; 76(5): 355-68

AB: We retrospectively studied the prevalence, histologic features,

clinical correlations, and long-term outcome of the intrarenal vascular

lesions of lupus nephritis (LN) in a series of 169 renal biopsies performed

between 1980 and 1994 in 132 patients with systemic lupus erythematosus.

The most common vascular lesions were nonspecific sclerotic changes, found

in 37% of the biopsies (24% if only the cases with moderate to severe

changes are considered). The other common vascular lesions were

"immunoglobulin microvascular casts," found in 24% of the biopsies.

Vasculitis and thrombotic microangiopathy were rare lesions and were seen

in only 4 (2.4%) and 1 (0.6%) cases, respectively. Isolated sclerotic

vascular changes were present in biopsies from older patients with a longer

duration of LN, compared with the group with no vascular lesions, and were

associated with a significantly higher prevalence of hypertension. Overall,

however, the long-term renal and patient survival of this group did not

differ significantly from that of the patients without vascular changes.

Immunoglobulin microvascular casts (IMCs) ("lupus vasculopathy") were

characterized by the presence of immunoglobulin deposition within the

glomerular capillaries and small arterioles. In the present study we

extensively investigated the morphologic and immunologic features of this

lesion. The lesions were notable for the absence of endothelial or parietal

vascular lesions and of fibrin, platelets, and leukocytes, which indicates

that thrombosis is not involved in the vascular obstruction. According to

our data immunoglobulin precipitation in the microvasculature seems to play

a central role in the pathogenesis of this lesion, which is why we propose

the term "immunoglobulin microvascular casts." In general, IMCs were

associated with the most severe and active forms of diffuse proliferative

lupus nephritis (World Health Organization [WHO] class IV). However our

data show that, in contrast to previous studies, the long-term outcome of

patients with IMCs is not worse than that of other patients with class IV

LN. It may even be somewhat better, suggesting that this type of lesion may

reverse with immunosuppressive therapy. In addition, we did not find any

association between the presence of IMCs and the lupus anticoagulant, IgG

anticardiolipin antibodies, or extrarenal vascular manifestations.

Concerning vasculitis and thrombotic microangiopathy, our results confirm

that their occurrence is quite rare in-lupus nephritis. The outcome of our

4 patients with vasculitis was not particularly poor, which could be

related to early and/or aggressive treatment. Taken as a whole, our data

confirm that the presence of active and severe forms of diffuse

proliferative LN (WHO class IV) carries a worse prognosis compared with the

other forms of LN. In our study, and in agreement with previous reports

(23), the long-term renal survival of patients with class IV LN was

significantly worse than that of patients with other forms of LN, with a

10-year renal survival of 70% compared with 85%, respectively. However our

data do not support the conclusions of some previous studies that the

presence of intrarenal vascular lesions is a marker of poor renal prognosis

in lupus nephritis. More precisely, our data show that the somewhat poorer

renal outcome observed in patients with IMCs is related to the fact that in

most cases these lesions are associated with class IV lupus nephritis, and

not related to the presence of the vascular lesion per se.

=====================================================================

35.) Antibodies to C1q in systemic lupus erythematosus: characteristics and

relation to Fc gamma RIIA alleles.

=====================================================================

AU: Haseley-LA; Wisnieski-JJ; Denburg-MR; Michael-Grossman-AR; Ginzler-EM;

Gourley-MF; Hoffman-JH; Kimberly-RP; Salmon-JE

SO: Kidney-Int. 1997 Nov; 52(5): 1375-80

AB: Autoantibodies to the collagen-like region of the first complement

component (C1qAB) are found in patients with systemic lupus erythematosus

(SLE), particularly those with renal disease. In a cohort of 46 SLE

patients with diffuse proliferative glomerulonephritis, we found declining

C1qAB titers in 77% of treatment responders and in only 38% of treatment

non-responders (P < 0.03). To further characterize this autoantibody, we

tested 240 SLE patients for the presence of C1qAB. Positive titers were

found in 44% of patients with renal disease and 18% of patients without

renal disease (chi2 P < 0.0003). Analysis of IgG subclass revealed IgG2

C1qAB alone in 34%, IgG1 C1qAB alone in 20%, and both IgG1 and IgG2 in 46%

of patients. Fewer than 10% of patients had measurable titers of IgG3 or

IgG4 C1qAB. The pathogenic role of these IgG2-skewed C1qAB may relate to

impaired immune complex clearance by the mononuclear phagocyte system: IgG2

antibodies are efficiently recognized by only one IgG receptor, the H131

allele of Fc gamma RIIa (Fc gamma RIIa-H131). In contrast, Fc gamma

RIIa-R131, which is characterized by minimal IgG2 binding, has recently

been associated with lupus nephritis. In our C1qAB positive patients, the

presence of Fc gamma RIIA-R131 was associated with an increased risk for

renal disease. Autoantibodies to C1q may have pathogenic significance in

SLE patients with genetic defects in the ability to clear IgG2 containing

immune complexes.

=====================================================================

36.) Heredity and systemic lupus erythematosus: dissecting a complex genetic

=====================================================================

disease.

AU: Shaver-TS; Harley-JB; Moser-KL

SO: Kans-Med. 1997 Winter-Spring; 97(3-4): 18-22

AB: The etiology of SLE appears to be exceedingly complex and possibly

heterogeneous, with genetics and environment both making substantial

contributions. A schematic representation of potential mechanisms is

depicted in Figure 2. We may not fully understand the pathogenesis of this

disease until we unravel the relative contributions of each component to

the development of SLE. While genetic mechanisms involved in SLE remain

obscure, we now have available elegant laboratory techniques for analysis

of genetic loci as well as computer technology which permits simulation and

analysis of the transmission of complex genetic traits among multiple

families and demographic groups. What remains is the painstaking task of

collecting families multiplex for SLE and analyzing multiple sets of

clinical, serologic, and genetic data within and between these pedigrees.

Such studies are currently underway and will hopefully increase

understanding of this enigmatic and complex autoimmune disorder.

=====================================================================

37.) Massive uncomplicated vascular immune complex deposits in the kidney of a patient with systemic lupus erythematosus.

=====================================================================

AU: Takazoe-K; Shimada-T; Nakano-H; Kawamura-T; Utsunomiya-Y; Kanai-T;

Kitajima-T; Yamaguchi-Y; Joh-K; Mitarai-T; Sakai-O

SO: Clin-Nephrol. 1997 Sep; 48(3): 195-8

AB: The case of a patient with systemic lupus erythematosus (SLE) is

reported which was accompanied by renal dysfunction and massive vascular

immune deposits in the kidney without active glomerular lesions. The renal

biopsy showed arterioles and small arteries with circumferential periodic

acid-Schiff (PAS) and Masson trichrome-positive homogenous material in the

subendothelial area in the absence of thrombotic, necrotizing or

inflammatory lesions. Immunofluorescence and electron microscopy

examination demonstrated immune deposits in the vascular walls. Glomeruli

showed only minor abnormalities with a trend to collapse. There was no

improvement in renal dysfunction over a 4-year period until the patient's

death, despite steroid therapy producing a decrease in disease activity.

The autopsy showed similar vascular changes to those seen in the biopsy,

however; glomeruli were either sclerotic or showed a trend to collapse.

Massive uncomplicated vascular immune complex deposition without active

glomerular lesions is rare. The present case indicates that this type of

lupus vasculopathy may be a prognostic factor for the loss of renal

function in SLE mediated by hemodynamic glomerular injury.

=====================================================================

38.) [Oral manifestations in patients with systemic lupus erythematosus]

=====================================================================

AU: Meyer-U; Kleinheinz-J; Gaubitz-M; Schulz-M; Weingart-D; Joos-U

SO: Mund-Kiefer-Gesichtschir. 1997 Mar; 1(2): 90-4

AB: Forty-six patients with systemic lupus erythematosus underwent thorough

dental examination to determine the frequency and severity of oral lesions

and periodontal diseases. According to clinical criteria, disease was

classified as severe (n = 26) or less severe (n = 20). The overall rate of

mucosal involvement in the studied patients was 48%-from 54% in patients

with severe disease, 40% in those with less severe disease. Patients with

severe disease were found to have a higher rate of tooth loss and an

increased rate of gingival inflammation. The severity of periodontal

lesions correlated with alterations in the immunoglobulin pattern,

particularly with an increase in gamma-immunoglobulins. Thus it is

suspected that complex immunodysregulation in combination with

immunosuppressive therapy is responsible for the high rate of oral and

periodontal lesions in patients with systemic lupus erythematosus.

=====================================================================

39.) Vasculitis and bacteraemia with Yersinia enterocolitica in late-onset

systemic lupus erythematosus.

=====================================================================

AU: Gauthier-T; So-AK

SO: Br-J-Rheumatol. 1997 Oct; 36(10): 1122-4

AB: We report a case of Yersinia enterocolitica 0.9 septicaemia

complicating systemic lupus erythematosus in an elderly male patient. The

infection gave rise to digital vasculitis, fevers and general malaise on

top of pre-existing articular symptoms. Features of Yersinia septicaemia

may mimic some of the signs of lupus.

=====================================================================

40.) Neoral--new cyclosporin for old?

=====================================================================

AU: Somerville-MF; Scott-DG

SO: Br-J-Rheumatol. 1997 Oct; 36(10): 1113-5

AB: Cyclosporin A is now well established as an effective second-line drug

to treat rheumatoid arthritis. In April 1995, the microemulsion-based

formulation of cyclosporin (Neoral) was introduced based on its increased

bioavailability at 'no extra cost'. There may have been concerns that with

increased bioavailability of Neoral, some patients might experience

increased toxicity, particularly if transferring from Sandimmun to Neoral

at the same dose. We describe our experience of 51 patients treated with

Neoral--39 with rheumatoid arthritis, six with psoriatic arthritis and the

remainder with a variety of diseases, including Behcet's, systemic lupus

erythematosus and juvenile chronic arthritis. All patients continued their

other medication including non-steroidal anti-inflammatory drugs and

analgesics. Five continued low dose prednisolone (average 7.5 mg per day)

all patients were monitored for safety and efficacy throughout their

treatment according to standard protocol. Five patients were enrolled in a

study of efficacy and safety where the dose of cyclosporin was reduced to

2.5 mg/kg/day at the time of conversion, i.e. to Neoral 2.5 mg/kg/day; 19

patients were converted dose for dose, cyclosporin A dose range 2.5-4

mg/kg/day converted to Neoral dose range 2.5-4 mg/kg/day and 27 patients

started Neoral de novo. We conclude that cyclosporin is a useful disease

modifying anti-rheumatic agent, and our experience suggests that the new

formulation, Neoral, has a similar safety and efficacy profile to the

original preparation (Sandimmun). Neoral was relatively easy to manage and

we noted a slight reduction in dose when compared to Sandimmun. With dose

adjustments over 18 months the mean dose for patients with RA fell from 3.2

to 2.7 mg/kg/day and of the 27 patients starting Neoral de novo only seven

required an increased dose above 2.5 mg/kg/day in order to establish efficacy.

=====================================================================

41.) Successful therapy with danazol in refractory autoimmune

thrombocytopenia associated with rheumatic diseases.

=====================================================================

AU: Blanco-R; Martinez-Taboada-VM; Rodriguez-Valverde-V; Sanchez-Andrade-A;

Gonzalez-Gay-MA

SO: Br-J-Rheumatol. 1997 Oct; 36(10): 1095-9

AB: The objective was to assess the efficacy of therapy with danazol in

refractory immune thrombocytopenia associated with different rheumatic

diseases. Patients with severe immune thrombocytopenia (platelet counts <

40 x 10(9)/l) with a bone marrow biopsy showing megakaryocytes in normal or

increased number and normal morphology were included if they fulfilled at

least one of the following criteria: (a) thrombocytopenia refractory to

prednisone (> or = 1 mg/kg/day during > or = 4 weeks); (b) patients

requiring an unacceptably high dose of prednisone for > 2 months

(prednisone dose > or = 20 mg/day); (c) no response to at least another

drug besides corticosteroids. Other causes of thrombocytopenia were

excluded. They were treated with danazol (100-200 mg q.i.d.) and followed

for at least 12 months. Four patients diagnosed with systemic lupus

erythematosus, two with rheumatoid arthritis and one with primary

antiphospholipid syndrome met the inclusion criteria. All of them achieved

acceptable platelet counts within the first 4 weeks of danazol therapy that

allowed the prednisone dosage to be tapered. No important side-effects

related to danazol therapy were observed. Danazol therapy seems to be a

useful and well-tolerated treatment for refractory immune thrombocytopenia

associated with different rheumatic diseases.

=====================================================================

42.) Predisposing factors in sulphasalazine-induced systemic lupus

erythematosus.

=====================================================================

AU: Gunnarsson-I; Kanerud-L; Pettersson-E; Lundberg-I; Lindblad-S; Ringertz-B

SO: Br-J-Rheumatol. 1997 Oct; 36(10): 1089-94

AB: The aim of this study was to define predisposing factors in patients

with sulphasalazine-induced systemic lupus erythematosus (SLE). Eleven

patients with onset of SLE or SLE-like syndromes during sulphasalazine

treatment are reported. Before the onset of SLE, five of the patients

suffered from rheumatoid arthritis (RA), one from psoriatic arthropathy

(PsoA), two from juvenile chronic arthritis (JCA) and three from ulcerative

colitis (UC). At the time of diagnosis of drug-induced SLE, analysis of

antinuclear antibodies (ANA), anti-double-stranded DNA antibodies

(anti-dsDNA), anti-histone antibodies (anti-histones), acetylator status of

the enzyme N-acetyltransferase 2 (NAT2) and HLA classification were

performed. All patients were anti-DNA positive at disease onset and were

determined to be slow acetylators. HLA A1 occurred in 4/10 patients, B8 in

5/10. HLA DR 3 was represented in one patient and DR 3(17) in five

patients. The DQA1* 0501 allele was observed in 7/10 patients and DQB1

0201* in 6/10. Persistent SLE and development of nephritis were noted in

patients with long duration of treatment and high cumulative dose of

sulphasalazine (> 1000 g). In sulphasalazine-induced SLE, slow acetylator

genotype and HLA haplotypes associated with idiopathic SLE seem to predict

disease induction. Further, as the risk of developing persistent SLE and

nephritis increases with long-standing sulphasalazine medication, it is of

importance to monitor the patients with regard to signs of SLE during the

entire treatment period.

=====================================================================

43.) Clinical features of lupus myositis versus idiopathic myositis: a

review of 30 cases.

=====================================================================

AU: Garton-MJ; Isenberg-DA

SO: Br-J-Rheumatol. 1997 Oct; 36(10): 1067-74

AB: Myositis is a rare but well-recognized complication of systemic lupus

erythematosus (SLE). It is reputed to be milder than primary myositis in

terms of morbidity and treatment response. This study compares clinical and

laboratory features of idiopathic inflammatory myositis in patients with

and without evidence of SLE overlap. We performed a case note review of 30

patients with probable or definite polymyositis/dermatomyositis of whom 11

also had definite or probable SLE. Lupus patients were slightly younger at

diagnosis than those with primary disease, and more likely to be female. At

presentation, quadriceps strength (expressed as a percentage of expected)

was significantly reduced in both the lupus (48.9%; 95% CI 29.0-70.4%) and

primary (52.0%; 95% CI 43.6-59.4%) myositis groups, and serum creatine

phosphokinase (expressed as a multiple of the upper limit of normal) was

significantly elevated (11.2; 95% CI 5.3-29.1 vs 10.7; 95% CI 6.1-17.6).

During a mean (S.D.) follow-up period of 7.4 (4.1) yr, both groups tended

to follow either a relapsing and remitting, or a chronic persistent course,

and when last seen quadriceps muscle strength remained significantly

depressed. One of the lupus patients and two of the primary myositis

patients died due to direct complications of the disease, and one further

death was attributable to a complication of therapy. Our results suggest

that lupus myositis is often as severe as primary disease and should be

treated with equal vigour.

=====================================================================

44.) Serological characteristics of systemic lupus erythematosus from a

hospital-based rheumatology clinic in Kuwait.

=====================================================================

AU: al-Mekaimi-A; Malaviya-AN; Serebour-F; Umamaheswaran-I; Kumar-R;

al-Saeid-K; Sharma-PN

SO: Lupus. 1997; 6(8): 668-74

AB: Thirty-one consecutive patients with SLE were screened for antinuclear

antibody (ANA), anti-DNA antibodies, extractable nuclear antigen antibodies

(anti-ENAs) including anti-Sm, anti-RNP, anti-SSA (anti-Ro), anti-SSB;

(anti-La), anti-Scl-70, rheumatoid factor (RF), C-reactive protein (CRP),

C3 and C4 levels, anti-cardiolipin antibodies (aCL), biologically false

positive serological test for syphilis (BF-STS) using VDRL test and Coombs'

test. The age of the patients ranged from 11 to 52 year with a median of 29

year; female to male ratio of 5:1. There were 21 Kuwaitis, four Egyptians,

three from the Indian subcontinent, two Filipinos and one Syrian. Main

clinical categories of SLE were: mild cutaneous SLE in 12 (38.7%), clinical

antiphospholipid syndrome (APS) secondary to SLE in 8 (25.8%),

haematological manifestations of SLE in 5 (16.1%), renal lupus in four

(12.9%), neuropsychiatric in three (9.7%), others (6.4%). Clinical features

overlapped in several patients. ANA was positive in 96.8% (mean value

891.61 units/ml), anti-DNA in 35.5% (mean value 56.4 units) that was lower

than expected and could be due to selection bias as the patients were from

a rheumatology clinic, anti-ENA in 42%, anti-Sm 13% that was lower than

other non-Caucasian populations, anti-RNP 13%, anti-SS-A in 35.5%,

anti-SS-B in 19.4%, Scl-70 in 13%, CRP in 71% (moderate 58%, very high

13%); C3 mean 1.52 mg/ml (3.2% low levels), C4 mean 0.35 mg/ml (32% low

levels), anticardiolipin mean GPL 35.35 units (high 58%), mean MPL 10.61

units (high 26%), BF-STS in 6%, Coombs' test in 6%, RF positive in 36%. The

only significant positive clinical associations observed were those of

renal involvement with anti-DNA antibodies (P = 0.042), and clinical

antiphospholipid antibody syndrome with aCL antibodies (P = < 0.05).

=====================================================================

45.) Elevated anticardiolipin antibodies in autoimmune haemolytic anaemia

irrespective of underlying systemic lupus erythematosus.

=====================================================================

AU: Lang-B; Straub-RH; Weber-S; Rother-E; Fleck-M; Peter-HH

SO: Lupus. 1997; 6(8): 652-5

AB: In patients with systemic lupus erythematosus (SLE) and concomitant

Coombs positive autoimmune haemolytic anaemia (AIHA) anticardiolipin

antibodies (aCL) are found more frequently and at higher titres than in SLE

patients without AIHA. In order to assess if aCL elevation is primarily

associated with the underlying SLE, or with AIHA itself, we examined AIHA

patients with and without underlying SLE for the presence of aCL. aCL (IgG

and IgM) were determined by ELISA in 74 SLE patients without AIHA, 22 SLE

patients with AIHA, 50 patients with idiopathic AIHA (warm-reactive

autoantibodies), 52 patients with idiopathic AIHA (cold-reactive

autoantibodies) and 50 healthy controls. The mean IgG and IgM aCL titres in

SLE patients without AIHA (IgG 37.0 U/ml, IgM 8.9 U/ml) were significantly

elevated compared with the values in healthy controls (IgG 9.1 U/ml, IgM

3.2 U/ml; P < 0.005). The mean aCL levels in SLE patients with AIHA (IgG

53.2 U/ml, IgM 28.2 U/ml) were higher than in SLE patients without AIHA (P

= 0.09 for IgG, P < 0.005 for IgM). Interestingly, the mean aCL levels of

patients with idiopathic AIHA (warm-reactive autoantibody type: IgG 29.2

U/ml, IgM 19.3 U/ml; cold-reactive autoantibody type: IgG 27.4 U/ml, IgM

18.9 U/ml) were also significantly elevated compared with healthy controls

P < 0.001). As aCL are elevated not only in SLE (with and without

concomitant AIHA) but also in idiopathic AIHA it can be speculated that aCL

are involved in the pathomechanism of autoantibody-induced erythrocyte

destruction per se irrespective of an underlying SLE.

=====================================================================

46.) A young woman with SLE: diagnostic and therapeutic challenges.

=====================================================================

AU: McDonagh-JE; Khamashta-MA; Menon-S; Rahman-A; Isenberg-DA

SO: Lupus. 1997; 6(8): 633-7

AB: The presence of antiphospholipid antibodies, especially of the IgG

isotype and in high titre, is associated with additional complications in

patients with SLE. The thrombocytopaenia and cerebral events in the patient

described are likely to have been linked to her lupus anticoagulant.

However, the antibody and anticardiolipin antibodies are not necessarily

synonymous and indeed we did not detect anticardiolipin antibodies in the

patient, although her sister had them. It is likely that they represent

overlapping sets of immunoglobulins. The production and analysis of further

such antibodies, as described in this review, is awaited urgently. Much

effort is also being expanded on identifying the precise targets for these

antibodies. In a very recent report Horkko et al have shown that these

antibodies may be directed against epitopes of oxidized phospholipids. The

management of patients with complex disorders such as described here remain

a challenge, although in the short term the patient's major locomotor,

neurological, dermatological and haematological problems have been

controlled. Long-term problems including impaired fertility and

osteoporosis remain to be faced.

=====================================================================

47.) Binding characteristics of SLE anti-DNA autoantibodies to modified DNA

analogues.

=====================================================================

AU: Arjumand-S; Moinuddin; Ali-A

SO: Biochem-Mol-Biol-Int. 1997 Oct; 43(3): 643-53

AB: Native calf thymus DNA was modified with furocoumarins and reactive

oxygen species (ROS). The modifications were probed by UV & Fluorescence

spectroscopy, thermal denaturation and hydroxyapatite chromatography. In CD

spectroscopy changes in the ellipticity of the DNA molecule were observed

as a result of modification. The binding specificity of naturally occurring

anti-DNA antibodies with modified DNA molecules was assessed by ELISA and

quantitative precipitin titration. The affinity of anti-DNA antibodies for

modified conformers was found to be quite high.

=====================================================================

48.) Circulating interleukin-6 type cytokines in patients with systemic

lupus erythematosus.

=====================================================================

AU: Robak-E; Sysa-Jedrzejowska-A; Stepien-H; Robak-T

SO: Eur-Cytokine-Netw. 1997 Sep; 8(3): 281-6

AB: We investigated the serum concentration of interleukin-6 (IL-6) and the

IL-6 family of cytokines (leukemia inhibitory factor (LIF), oncostatin M

(OSM) and ciliary neurotrophic factor (CNTF) using an enzyme-linked

immunosorbent assay (ELISA) in 64 patients with systemic lupus

erythematosus (SLE) and 15 healthy controls. We also examined a possible

association between the serum levels of these proteins and SLE activity as

well as correlations between the IL-6 concentration and the levels of LIF,

OSM and CNTF. IL-6 was detectable in all 64 patients with SLE and normal

individuals, and the level of this cytokine was significantly higher in

patients than in the control group (p < 0.002 ). LIF, OSM and CNTF were

detectable in 9 (14.1%), 6 (9.4%) and 51 (78%) patients, respectively, and

undetectable in the majority of healthy individuals. We found positive

correlation between the serum concentrations of IL-6, LIF, OSM and CNTF and

SLE activity. IL-6 and OSM serum levels were also correlated but not IL-6

and LIF or CNTF. In conclusion, an increase in the serum levels of IL-6

and, to a lesser extent of LIF, OSM and CNTF concentrations may be useful

markers for SLE activity.

=====================================================================

49.) [Osteonecrosis in systemic lupus erythematosus. Report of 3 cases]

=====================================================================

AU: Marron-A; Formiga-F; Valverde-J; Mitjavila-F; Pac-M; Moga-I

SO: An-Med-Interna. 1997 Jun; 14(6): 307-9

AB: We present three cases of patients with systemic lupus erythematosus

(SLE) and osteonecrosis or avascular necrosis (AV). Although, the

pathogenesis of osteonecrosis is controversial and multifactorial, the

glucocorticoids therapy is the most important factor contributing to the

lesion. We report the clinical presentation of the three patients. We

comment the characteristics of AV, the diagnosis and the treatment of this

uncommon complication in SLE patients.

=====================================================================

50.) Association of an insertion polymorphism of angiotensin-converting enzyme gene with the activity of systemic lupus erythematosus. 

=====================================================================

Author 

Sato H; Akai Y; Iwano M; Kurumatani N; Kurioka H; Kubo A; Yamaguchi T; Fujimoto T; Dohi K 

Address 

First Department of Internal Medicine, Nara Medical University, Kashihara, Japan. 

Source 

Lupus, 7(8):530-4 1998 

Abstract 

Systemic lupus erythematosus (SLE) shows various clinical manifestations, which are characterized by inflammation in many different organ systems. The cause of SLE is still unclear; however, the immunological abnormalities are considered to be responsible for the pathogenesis of SLE. As angiotensin I-converting enzyme (ACE) has been reported to be associated with various immunological phenomena, we investigated the correlation between insertion (I)/deletion (D) polymorphism of the ACE gene and the disease activity of SLE. Ninety-three patients with newly diagnosed SLE were enrolled in this study. ACE genotype was determined by the polymerase chain reaction (PCR). We measured serum levels of anti-double-stranded (ds) DNA antibody (Ab) and serum levels of total complements (CH50) as the parameter for lupus activity. Moreover, we evaluated the clinical disease activity by calculating SLE disease activity index (SLEDAI). Individuals with II genotype showed a significant increase in SLE activity. Patients with the ACE II genotype showed a higher serum level of anti-dsDNA Ab (14.3 IU/ml (5.475, 74.6, median (25th centile, 75th centile)) than those with the DD genotype (4.65IU/ml (4.05, 6.8)) (P<0.01). Moreover, patients with the 11 genotype also showed lower levels of serum CH50 than those with the DD genotype (P < 0.01). Patients with the II1 or DI genotype had significantly higher SLEDAI score than those with the DD genotype (P < 0.01). These results suggest that the ACE genotype could be associated with the disease activity of SLE. ACE insertion polymorphism might be used as one of predictive factors for the activity of lupus. 

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DATA-MÉDICOS/DERMAGIC-EXPRESS No (53) 07/05/99 DR. JOSE LAPENTA R. 

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Produced by Dr. José Lapenta R. Dermatologist
Venezuela 1.998-2.024

Producido por Dr. José Lapenta R. Dermatólogo Venezuela 1.998-2.0024

Tlf: 0414-2976087 - 04127766810