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PubMedDate not supplied

Disease activity in systemic lupus erythematosus: report of the Consensus Study Group of the European Workshop for Rheumatology Research. I. A descriptive analysis of 704 European lupus patients. European Consensus Study Group for Disease Activity in SLE.

Using a detailed questionnaire, the cumulative historical and current demographic, clinical and serological data on 704 SLE patients from 29 European centres and 14 countries have been assessed. Ninety-three percent of the patients were Caucasian and the female/male ratio was 10:1. Analysis of the cumulative incidence showed that arthralgia/arthritis (94%), rash (69%), Raynaud's phenomenon (49%), serositis (44%) and renal disease (38%) were the most frequent clinical manifestations. Virtually all the patients (98%) were antinuclear antibody positive, while anti-ds-DNA antibodies (76%), hypocomplementaemia (71%) and anti-Ro(SSA) antibodies (35%) were frequent serological abnormalities. Whilst much of this data is in line with previous reports, it is notable that renal, lung, and central nervous system involvement and the frequency of rheumatoid factor, anti-Sm and anti-RNP antibodies were much lower than in most comparable series in the United States. We assume that ethnic differences and the greater present awareness of lupus could explain this variations. Low dose corticosteroids, non-steroidal anti-inflammatory drugs and anti-malarials were used to treat over half of the patients, 75% of whom were between 15 and 55 years of age. This report offers a useful overview of lupus both clinically and serologically in Europe in the 1990's.

Adolescent↗
PubMedDate not supplied

Disease activity in systemic lupus erythematosus: report of the Consensus Study Group of the European Workshop for Rheumatology Research. III. Development of a computerised clinical chart and its application to the comparison of different indices of disease activity. The European Consensus Study Group for Disease Activity in SLE.

In the first phase of this study, a data-base containing clinical and laboratory findings of 704 patients with systemic lupus erythematosus (SLE), originating from 29 centres and 14 countries, was used to assess the validity of 4 common indices of disease activity, SLAM, BILAG, SLEDAI and SIS. The physician's judgement of activity was assumed as the unique reference criterion (gold standard). Computer programmes were developed to calculate automatically the 4 activity indices; this computation appeared to correspond with manual computations in a sample of 60 appropriately selected cases. All 4 indices were closely correlated with each other (r in the range of 0.716 to 0.872), and with the physician's score (r in the range of 0.620 to 0.719). In the second phase of the study the activity index developed in part I (ECLAM) was prospectively validated, and its performance compared to that of the other scales, both as a single state index and as a transition index (i.e., its ability to assess disease activity at a single point in time and to detect variations in consecutive readings). A computer-assisted clinical chart was prepared for this purpose. This chart allowed us to calculate automatically all the indices. Two consecutive observation times (time 0, and time 1 three months later) were included in the study protocol. Data on 75 patients from 19 centres were collected, and each patient was observed twice. All the computed indices were closely correlated, both at time 0 (r ranging from 0.725 to 0.884), and at time 1 (r ranging from 0.607 to 0.833).(ABSTRACT TRUNCATED AT 250 WORDS)

Diagnosis, Computer-Assisted↗
PubMed2005

[Is the primary antiphospholipid syndrome a forerunner of SLE?].

INTRODUCTION: Antiphospholipid syndrome is a multi-organ autoimmune disorder, characterized by arterial and venous thrombotic events, and a well-defined group of recurrent foetal wasteage due to pathologic antibodies against phospholipids and protein co-factors. Antiphospholipid antibodies can be formed in primary antiphospholipid syndrome, but also in other conditions, most often in systemic lupus erythematosus. This may modify the outcome of lupus increasing the risk for thrombotic complications. Less is known about the outcome in primary antiphospholipid syndrome, whether it may precede the development of systemic lupus. AIMS: Authors hereby describe the case of four patients with primary antiphospholipid syndrome in whom the disease progressed to systemic lupus erythematosus. RESULTS: Lupus followed the primary antiphospholipid syndrome within around a three-year period. It was indicated by the appearance of different antinuclear autoantibodies and clinical complications, such as polyarthritis, nephritis and hematologic disturbances. All of the patients presented cerebrovascular accident as the thrombotic manifestation. All but one, were around forty years old, and had a milder form of lupus. Symptoms of the antiphospholipid syndrome determined the outcome. On the other hand, a typical lupus developed in the youngest patient. CONCLUSIONS: According to present cases antiphospholipid syndrome may be considered as the initiative phase of SLE, but APS being a separate entity also may associate to lupus. Present observations indicate the importance of follow-up the patients with APS by immunologic respect. Future prospective, multi-centre studies with larger number of cases are needed to provide further evidence on the fact that patients with APS may acquire other autoimmune disorder.

Adult↗
PubMed2005

Adjusted mean Systemic Lupus Erythematosus Disease Activity Index-2K is a predictor of outcome in SLE.

OBJECTIVE: To test the predictability of the adjusted mean Systemic Lupus Erythematosus Disease Activity Index-2K (AMS) for main outcomes in systemic lupus erythematosus (SLE), namely presence of damage, coronary artery disease (CAD), and avascular necrosis (AVN). METHODS: Included in this study are patients with regular followup from the University of Toronto Lupus Clinic. This was defined as a minimum of 3 visits and no absence exceeding 18 consecutive months. For each visit, AMS was evaluated. The ability of the AMS to predict each of the main outcomes was evaluated through time-dependent covariate survival analysis. Adjustments to the regression models were made to include other risk factors such as sex, age at diagnosis (AGE), SLEDAI-2K at presentation (SLEDAI), disease duration (DD), and use of corticosteroids, immunosuppressives (IM), or antimalarials (AM). RESULTS: Five hundred and seventy-five patients were included covering the period from 1970 to 2002. A total of 325 developed damage, 55 had CAD, and 68 had AVN. Presence of damage was not associated with sex, SLEDAI, or AM but was significantly associated with AMS, AGE, DD, and use of steroids or IM (all p < 0.001). CAD was not associated with SLEDAI or use of steroids or AM but with all other variables AMS (p = 0.046), sex (p = 0.009), AGE (p < 0.0001), DD (p < 0.0001), and IM (p = 0.035). Predictors of AVN were DD (p = 0.032) and IM (p < 0.0001) but not sex, AGE, use of steroids, AM, SLEDAI, or AMS. CONCLUSION: AMS is associated with the presence of damage and CAD. It is not associated with AVN.

Adolescent↗
PubMed2005

[Pregnancy in women with systemic lupus erythematosus (SLE)].

This paper presents the view of literature on pregnancy in systemic lupus erythematosus. It includes opinions on the risk of lupus exacerbation during the pregnancy. Despite much researches, it was not possible to give a precise answer to the question whether pregnancy induces increased rate of lupus flare. The women suffering from SLE face higher risk of fetal losses, preterm births or intrauterine growth retardations. However, if the pregnancy is properly planned and under interdisciplinary medical care, it is highly possible, besides exceptional cases, to give birth to a healthy child. Most of the authors do not advise against pregnancy with lupus patients.

Female↗
PubMedDate not supplied

Active human herpesvirus-6 (HHV-6) infection associated with Kikuchi-Fujimoto disease and systemic lupus erythematosus (SLE).

Histiocytic necrotizing lymphadenitis (Kikuchi-Fujimoto disease) is a well defined disorder primarily affecting young adults. The cause of this disease is still unknown. The authors report a case of a 37-yar old woman with Kikuchi-Fujimoto disease and systemic lupus erythematosus (SLE). Serologic testing for HHV-6 antibodies revealed an active infection. An excised cervical lymph node contained HHV-6 genome demonstrated by using in situ hybridization. Active HHV-6 infection should be considered in Kikuchi-Fujimoto disease.

Adult↗
PubMedDate not supplied

Anti-phosphotyrosine and anti-phosphoserine antibodies in SLE sera.

By using an ELISA method, we identified antiPTyr and antiPSer antibodies in the sera of some SLE patients. Afterwards, antiPTyr and antiPSer antibodies were purified by affinity chromatography on phosphotyramine-CNBr-Sepharose column and on phosphoethanolamine-CNBr-Sepharose, respectively, and the specificity of the purified antibodies was demonstrated by inhibition assays. The study pointed out a higher incidence of antiPTyr than anti PSer antibodies in the sera of these patients, which suggests that some "autoantigens" from membrane might be involved.

Antibodies↗
PubMed1991

[The efficacy of cyclophosphamide bolus therapy in a girl with SLE with a nephritic onset].

A case of glomerulonephritis as the initial clinical manifestation of SLE in a child is reported. Treatment with oral prednisone did not produce beneficial results either worth respect to the symptoms or the laboratory data. The association with intravenous Cyclophosphamide led to rapid improvement in clinical and serological patterns. Moreover this association has reduced the risk of end-stage renal failure with few serious complications, and no other side-effects.

Biopsy↗
PubMedDate not supplied

A study of synovial membrane of patients with systemic lupus erythematosus (SLE).

In this study, 30 percutaneous synovial knee biopsies were taken from patients with systemic lupus erythematosus (SLE) that had been diagnosed in accordance with ARA criteria. Acute articular involvement was present in 16 cases. The synovial membrane was then examined using routine histological techniques and by direct immunofluorescent methods. The most frequently observed histological picture was comprised of: a) synoviocyte hyperplasia, b) scarce inflammatory infiltrate, c) vascular proliferation, d) oedema and congestion, e) fibrinoid necrosis and intimal fibrous hyperplasia of blood vessels, g) presence of fibrin on the synovial surface and fibrin-like deposits in the chorion. It is concluded that immunofluorescence can be used in the characterization of typical cases but is not specific.

Adolescent↗
PubMed1991

[Normolipemic xanthoma developed on alopecia lesion on a SLE patient--histological study].

A 30-year-old SLE patient developed a xanthoma on her alopecia lesion. Histological examination showed typical lupus changes including immunofluorescence stainings. In addition, there were numerous xanthoma cells existed through the dermis, interlobular spaces of subcutaneous fat tissue, perivascular areas of small blood vessels, and subendothelial spaces and inside of intraluminal thrombosis of subcutaneous small arteries. These xanthoma cells contained granular materials in their cytoplasm. Histochemically, these materials are diastase resistant P.A.S. (+), immunoglobulin (+), Sudan BB (+), Sudan III (+), Nile blue (pinkish red), and yellowish orange autofluorescence suggesting that they are lipofuscin or lipid peroxidation products. Further histological findings showed destruction of sebaceus glands and peri-glandular++ infiltration of lymphocytes and histiocytes. These histiocytes contained phagocytic neutral lipid droplets in their cytoplasm. Very small lipid peroxides were also seen on surface and/or cytoplasm of infiltrating lymphocytes existing not only peri-lobular area but also intraluminal area of blood vessels. The marker profile of these infiltrating lymphocytes are B-cell predominant admixed with some CD8(+) T-cells. These data suggest that the mechanism of developing xanthoma is initiated by immune-complex deposition on basal lamina of sebaceus glands, followed by destruction of sebaceus glands by lympho-histiocytic cells infiltration with some antigen presenting and/or effector cells, and finally xanthoma cells were developed by phagocytosis of lipid peroxides caused by macrophage-derived oxygen radicals. Interestingly, our data suggest that the lipid peroxides, which may act as photosensitizer, may leave the skin and may enter the small vessels carried by lymphocytes. Furthermore the xanthoma cells may also enter the circulation through the small arteries.

Adult↗
PubMed1991

Vascular lesions in SLE.

The confusing array of manifestations resulting from multisystem involvement in SLE is due, in part, to the widespread involvement of blood vessels.

Humans↗
PubMed1990

SLE nephritis: an ultrastructural immunogold study to evaluate the relationship between immune complexes and the basement membrane components type IV collagen, fibronectin and heparan sulphate proteoglycans.

The nature of immune complexes and their relationship to the normal glomerular basement membrane (GBM) components type IV collagen, fibronectin, and heparan sulphate proteoglycans (HSPG) have been examined in the glomeruli of 7 cases of systemic lupus erythematosus (SLE) glomerulonephritis using an ultrastructural immunogold technique. In paraformaldehyde-fixed, Lowicryl resin-embedded tissue, the electron-dense deposits contained IgG, IgM, IgA, and C3 whether they were subepithelial, intramembranous, subendothelial, or mesangial and there was no particular relationship between the class of immunoglobulin and site of immune complex localization within the glomerulus. The normal GBM components type IV collagen, fibronectin, and HSPG were found within all the glomeruli, but did not have the same distribution. Type IV collagen and fibronectin were found predominantly on the inner aspect of the GBM and diffusely throughout the more central regions of the mesangial matrix. By contrast the HSPG was seen mainly on the outer aspect of the GBM and at the periphery of the mesangial matrix. In none of the cases were GBM antigens localized within the electron-dense deposits, results which suggest that autoantibodies to these GBM components may not play a role in the development of the glomerulonephritis.

Adult↗
PubMed1990

Naturally occurring anti-native DNA antibodies in SLE binding Z-DNA.

A high degree of specificity of circulating anti-DNA antibody for double stranded DNA in the sera of SLE patients was found, as compared to single stranded polymer. The antibody recognized brominated DNA, a polymer that appears to attain Z-conformation as indicated on the basis of UV absorption characteristics. The existence in native DNA of regions undergoing B----Z transition has been detected.

Antibody Specificity↗
PubMed1989

Myocardial infarction and antiphospholipid antibodies in SLE and related disorders.

The clinical and serological findings in 13 patients with myocardial infarction and antiphospholipid antibodies (the 'lupus anticoagulant', antibodies to cardiolipin, antibodies to phosphatidylethanolamine (one patient] seen by our unit and other units from 1984 to 1989, are presented (eight males and five females, ages ranging from 20 to 52 years). Five suffered myocardial infarction before the age of 30; four of these five were in their early 20s. Other risk factors such as excessive smoking (greater than 20 cigarettes a day) (two patients), long-term treatment with steroid (one) and use of oral contraceptives (one) were present. One patient had demonstrated a plasminogen activator deficiency and one a deficiency of protein C. Two patients developed myocardial infarction six to eight weeks after warfarin was discontinued for recurrent deep vein thrombosis. Six patients had SLE as defined by the revised 1982 criteria, three suffered from 'lupus-like' disease, while four patients conformed to a 'primary' antiphospholipid syndrome.

Adult↗
PubMed1989

[Phenotype analysis of peripheral blood T lymphocytes from SLE patients by flow cytometry].

Phenotype of OKT3, OKT4 and OKT8 markers on surface of peripheral blood T lymphocytes from 31 SLE patients were analyzed with a flow cytometer and the results were compared with those of T cells from 36 normal controls. The results showed that the rates of OKT3+ and OKT4+ cells were within the normal range, while that of OKT8+ cells (44.52 +/- 12.07%) was significantly higher than normal (30.39 +/- 6.06%, P less than 0.01). Besides, the OKT4+/OKT8+ ratio in patients (0.69 +/- 0.24) was lower than that of normal controls (1.06 +/- 0.28, P less than 0.01), 5/13 (38.5%) cases with kidney lesion showing obvious decrease of OKT4+/OKT8+ ratio, while 1/18 cases (5.56%) without kidney lesion also showing such decrease.

Adult↗
PubMed1989

[A case of overlap syndrome of PSS, SLE and Sjögren syndrome treated by cyclosporin A: an improvement of sclerosis of the skin].

A 26-year-old female patient complicated with PSS, SLE and Sjögren was treated by combination of medium doses of corticosteroid and cyclosporin A. On admission, she showed many abnormal laboratory findings such as increased serum gammaglobulin level, high anti-DNA antibody titer and low compliment level. This therapy improved not only these laboratory abnormalities but clinical symptoms including sclerosis of the skin and arthritis, without notable side effect due to either of these drugs for the following 10 months so far. On the other hand we haven't acknowledged the effect to internal involvement yet. Since there is no promising therapy to sclerosis of the PSS skin, it is suggested that cyclosporin A can be an effective agent for PSS and should be further evaluated.

Adult↗
PubMed1989

Kidney biopsy in SLE. I. A clinical-morphologic evaluation.

The relationship between renal morphology and clinical disease was analysed in 148 patients with SLE attending a lupus clinic. Patients were not selected for renal disease. Renal tissue was assessed according to the World Health Organization classification of lupus nephritis, the presence of active and chronic lesions was recorded and disease activity was measured according to a standard protocol. All sections of the classification were represented in this group of patients. Active and chronic lesions were more likely to occur among patients with Class III/IV (proliferative glomerulonephritis), than in any other category. Patients with Class III/IV biopsy were more likely to have evidence of clinical renal disease than patients in Class II (mesangial). However, almost half of the Class II patients had some evidence of renal disease, including elevated serum creatinine, as well as important non-glomerular lesions. Without biopsy they might have been thought to have proliferative lesions and been treated more aggressively. Two patients with proliferative glomerulonephritis had no clinical evidence of renal disease. Thus, at the time of biopsy results renal histological examination did not uniformly correlate with clinical renal disease.

Adolescent↗