Adult onset Still's disease and collapsing glomerulopathy: successful treatment with intravenous immunoglobulins and mycophenolate mofetil.
ABSTRACT In this Grand Round we present a 32-yr-old African man who became severely ill after a 5-month history of weight loss, pyrexia, arthralgia, sweats and rash. He went on to develop pericarditis, pericardial effusion with tamponade, hepatomegaly with abnormal liver function tests, lymphadenopathy, massive proteinuria and required ventilatory, circulatory and renal support. The differential diagnosis was adult onset Still's disease, systemic lupus erythematosus (SLE), infection and lymphoma. Primary infection and lymphoma were excluded and he was treated, with dramatic success, with intravenous immunoglobulins (i.v.IG). Subsequent renal biopsy excluded SLE but confirmed collapsing glomerulopathy. The proteinuria improved dramatically following treatment with mycophenolate mofetil. We discuss some of the difficult diagnostic and management issues raised by this patient and the different uses and mechanisms of action of i.v.IG.
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ABSTRACT: Six patients are reported with nephrotic syndrome and relatively rapid progression to irreversible renal failure. The renal histologic findings are unusual. The most striking changes are collapse of glomerular capillary loops, such as might occur with glomerular hypoperfusion; significant tubulointerstitial damage is also seen. These patients appear to have a clinicopathologic entity hitherto unreported. Its differentiation from other causes of progressive glomerular damage, and particularly from focal and segmental glomerulosclerosis, is discussed.American Journal of Kidney Diseases 02/1986; 7(1):20-8. · 5.29 Impact Factor
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ABSTRACT: The focus of this review is to summarize the mechanism and the adverse side-effects of Intravenous Immunoglobulin (IVIg) therapy, and to highlight the current cumulative experience of its use in the treatment and management of autoimmune and systemic inflammatory diseases. Detailed search of the literature was done. Studies involving only humans were considered for clinical evaluation. Animal studies were used only for understanding mechanisms of action. The NIH Consensus Conference of May 1990 and the Australian Society for Blood Transfusion of July 1993 were used for guidelines. Material was taken only from peer reviewed journals. It appears that IVIg may act by more than one mechanism of action. It is unclear whether the mechanism is different in different diseases and whether more than one mechanism may apply to any disease or clinical state. The incidence and gravity of serious side effects appears low. It is the mainstay of treatment of immune thrombocytopenia purpura and Kawasaki disease. IVIg is a safe and effective therapeutic modality that can be added to the repertoire of various agents used to treat autoimmune and systemic inflammatory diseases. Long-term prospective studies are needed to define indications, dose-schedules, duration of therapy, and influence on the clinical courses of chronic diseases better.Annals of allergy, asthma & immunology: official publication of the American College of Allergy, Asthma, & Immunology 03/1995; 74(2):119-28; quiz 128-33. · 3.45 Impact Factor
- The Lancet 03/1996; 347(8997):337. · 39.06 Impact Factor