[Diagnosis and therapy of systemic lupus erythematosus].
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A 33-year old housewife who developed BOOP in a flare-up stage of SLE was reported. She had been treated for Hashimoto's thyroidtis since the age of 18. When the patient was 21 years old, she noticed polyarthritis and subcutaneous nodules. She had a high titer of rheumatoid factor and a diagnosis of rheumatoid arthritis was made. At the age of 23, ulnar drift was noted despite the lack of subjective and objective signs of active articular inflammation. At the age of 25, she developed dry eyes and dry mouth, and at the age of 27, photosensitivity was experienced. At the age of 28, she was first admitted to our department and diagnosis of Sjögren's syndrome and SLE was made. Since then, she had been treated by corticosteroid and remained in a stable condition. In January 1991, she developed facial erythema, dry cough and edema of the lower extremities. Laboratory findings included profuse proteinuria, hypoalbuminemia, hyperlipidemia, elevated anti-DNA titer, depressed serum complements. Chest X-ray films showed a presence of patchy non-segmental infiltrates in both lung fields. TBLB specimen showed the characteristic findings of BOOP described by Epler at al. She had marked ulnar deviation at the MCP joints bilaterally and X-ray findings were consistent with Jaccoud's arthropathy. The pulmonary lesions and nephrotic syndrome responded to the combined therapy of prednisolone and immunosupressants. An occurrence of BOOP associated with flare of SLE indicates that immunological mechanisms might be involved for development of BOOP. Indeed, review of the literature shows that substantial numbers of patients with BOOP had clinical and serological overlapping findings with autoimmune diseases.(ABSTRACT TRUNCATED AT 250 WORDS)
Extensive studies in murine models of lupus nephritis have shown that cationic anti-DNA autoantibodies have nephritogenic potential. We have investigated whether cationic anti-DNA antibodies of IgG class are also produced in vivo in patients with active lupus nephritis. Antibodies against DNA in the sera from patients with SLE were purified by affinity chromatography on DNA-cellulose, followed by nonequilibrium pH gradient electrophoresis and SDS-PAGE. The highly cationic anti-DNA antibodies of IgG class were prominent in the nonequilibrium pH gradient electrophoresis-immunoblots of the antibodies from the patients with active lupus nephritis. Decreased proteinuria after successful treatment with prednisolone was associated with disappearance of the cationic anti-DNA antibodies in the circulation. The cationic anti-DNA antibodies did bind to heparan sulfate, which is a major glycosaminoglycan in glomerular basement membrane, much better than did neutral anti-DNA antibodies. The results suggest that the cationic anti-DNA autoantibodies may play a certain role in the development of lupus nephritis. Our study demonstrates the strong association between the presence of cationic anti-DNA antibody and the development of lupus nephritis in humans.
In the present study we have analyzed the prevalence and characteristics of the most relevant clinical and immunologic features in 1,000 patients with SLE. Several differences in the expression of the disease have been observed in relation to the patients' age at onset, sex, and autoantibody serology. The childhood-onset patients more often had malar rashes (55% vs 39%) and nephropathy (28% vs 15%) as presenting manifestations. During the evolution of the disease, these patients had an increased prevalence only of malar rash (79% vs 56%) and a lower prevalence of rheumatoid factor (6% vs 19%). The older-onset patients (age 50 or older) less often showed malar rash (21% vs 42%), arthritis (52% vs 71%), and nephropathy (3% vs 17%) as the first symptom. During the evolution of their disease, these patients had a decreased prevalence of malar rash (33% vs 60%), photosensitivity (29% vs 47%), arthritis (73% vs 85%), nephropathy (22% vs 41%), thrombosis (4% vs 15%), and anti-La antibodies (6% vs 20%), but an increased prevalence of sicca syndrome (33% vs 15%). Males more often had serositis (28% vs 16%) as a first symptom, but they presented with a lower prevalence of arthritis (74% vs 85%) during the evolution of the disease. The presence of ANA, a high titer of anti-dsDNA, rheumatoid factor, anti-ENA, and antiphospholipid antibodies also distinguished additional homogeneous SLE subsets of clinical significance.
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Serial immunological testing has been recently proposed for monitoring patients with lupus nephritis as routine serological tests have shown sub-optimal correlation with clinical status. To assess the value of urine cytology and urine sIL2R in the evaluation of patients with SLE, in particular those with lupus nephritis, we conducted a prospective double-blind study of 31 patients with SLE, during an 18-month period. A comparison of routine urinalysis with urine cytology and urine sIL2R was performed in 84 samples: 15 from patients without a history of renal involvement and 69 from patients with a history of renal involvement. A high urine cytology score (> or = 6), particularly in the presence of lymphoblasts, plasma cells or monocytes, was significantly associated with lupus nephritis in relapse. Urine sIL2R levels were significantly elevated during all SLE relapses, unrelated to the presence of renal involvement. Fifteen urine specimens were obtained at the time of a kidney biopsy: 9 with active lesions and 6 with inactive renal disease. UC score was 2.0 +/- 1.89 for those with absent activity, 8.4 +/- 3.4 for mild activity and 11.0 +/- 2.4 for moderate/severe activity (p < 0.001 between active vs inactive disease). No urinalysis parameter alone permitted distinguishing the degree of renal disease activity. In the subgroup of patients with renal disease urinalysis was overall less accurate than urine cytology or urinary sIL2R levels for predicting renal disease activity defined by biopsy. Urine cytology and urine sIL2R proved to be reliable measures of lupus activity.
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A 22 year-old woman with a seven year history of (SLE) was readmitted because of oliguria, edema, dyspnea and arterial hypertension. She had a previous biopsy diagnosis of focal glomerulonephritis, (WHO III b), and had been treated with immunosuppressors and steroids. Laboratory data showed lupus activity, AHM with thrombocytopenia, nephrotic-range proteinuria and renal failure. A second renal biopsy was performed showing diffuse proliferative nephritis, (WHO IV), in association with noninflammatory necrotizing vasculopathy with luminal obliteration. She started with hemodialysis and was subsequently treated with methylprednisolone pulses, plasmapheresis, cyclophosphamide and oral steroids. During the inpatient period, she had generalized seizures, acute lung injury and pulmonary hemorrhage. These complications, the AHM and the thrombocytopenia receded totally. Renal function was never resumed. We emphasize that this association of diffuse proliferative nephritis with noninflammatory necrotizing vasculopathy is not infrequent and has a poor renal prognosis. The AHM with thrombocytopenia was interpreted as secondary to endothelial cell damage due to vasculopathy.
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We identified clinical predictors of lupus nephritis presenting more than 6 months after the diagnosis of lupus in a cohort of 335 lupus patients. Almost 24% (80/335) of patients developed nephritis more than 6 months after the diagnosis of lupus. Using a Cox proportional hazards model, we found hypertension, thrombocytopaenia and leukopaenia to be associated with lupus nephritis presenting more than 6 months after diagnosis, with adjusted relative risks of 2.5 (95% CI 1.3 to 4.7), 4.3 (95% CI 1.7 to 10.8) and 3.2 (95% CI 1.7 to 6.2) respectively. In this cohort, hypertension, thrombocytopaenia and leukopaenia were associated with lupus nephritis presenting more than 6 months after the diagnosis of lupus.
OBJECTIVE: Although systemic lupus erythematosus of varying severity has been seen in our clinics, there is no information regarding the prevalence of the disease among Saudi nationals. We conducted this study to determine the prevalence of systemic lupus erythematosus in Al-Qaseem region of the Kingdom of Saudi Arabia. METHODS: A community survey of towns and villages in the Al-Qaseem area of central Saudi Arabia was conducted in 3 phases to determine the prevalence of systemic lupus erythematosus in the region. RESULTS: Of the 10,372 studied, 2 cases of systemic lupus erythematosus were identified using the criteria set for the diagnosis of systemic lupus erythematosus by the American College of Rheumatology. Based on that, the prevalence of systemic lupus erythematosus was estimated to be 19.28 per 100,000 population in the region. CONCLUSION: The estimated prevalence of systemic lupus erythematosus in Al-Qaseem area is similar to that found in western countries.
Severe mitral valve regurgitation due to systemic lupus erythematosus is a rare cause of valvular heart disease, necessitating valve surgery. Currently, there are 36 case reports in the world medical literature of mitral valve replacement or repair in patients who have lupus. The current trend in mitral valve surgery is toward anatomic valve repair. In patients who have systemic lupus erythematosus, however, valve repair often leads to repeat surgery and valve replacement. We report the cases of 5 patients with lupus and severe mitral valve regurgitation who underwent mitral valve surgery. In 3 of these patients, replacement with a mechanical prosthetic mitral valve was performed with good long-term results. In the other 2 patients, mitral valve repair was performed, but only 1 of the repairs was successful. The 2nd patient required subsequent replacement with a mechanical valve. To our knowledge, this report of 5 patients is the largest series of mitral valve surgery in patients with lupus. These results, along with a review of the literature, suggest the superiority of mechanical prosthetic valve replacement to repair in patients who have systemic lupus erythematosus.
The assessment of disease activity in systemic lupus erythematosus (SLE) is a task faced by clinicians in every day care, but it is also required for clinical research and in randomised controlled trials. It is crucial to distinguish disease activity from infection, chronic damage and co-morbid disease. Over the past 20 years, many indices have been developed to objectively measure lupus disease activity and several of these have been validated. The most widely used indices are the British Isles Lupus Assessment Group (BILAG) index, the European Consensus Lupus Activity Measurement (ECLAM), the Systemic Lupus Activity Measure (SLAM), the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) and the Lupus Activity Index (LAI). All these indices have been validated and have excellent reliability, validity and responsiveness to change. In addition to the assessment of disease activity, the evaluation of damage using the validated SLICC/ACR damage index and health-related quality of life is advised for clinical research.