[Academician V.A. Nasonova's role in the study of systemic vasculitides].

Article is dedicated to academician V.A.Nasonova contribution to the development of clinic, diagnostics and treatment of systemic vasculitides].

Use of muscle biopsies for diagnosis of systemic vasculitides.

OBJECTIVE: Few studies have investigated the use of muscle biopsies (MB) for the diagnosis of systemic vasculitides (SV). We aimed to evaluate the diagnostic use of MB in this condition. METHODS: We reviewed 310 consecutive MB performed in our center between 2000 and 2008 and correlated them with clinical data from the corresponding patients. Thirty-one of the patients, representing a total of 33 MB, were diagnosed with active SV. MB were considered positive when they demonstrated either necrotizing vasculitis or nonnecrotizing vasculitis. RESULTS: Twenty-two of the 33 MB were positive (sensitivity of 66.7%), with necrotizing vasculitis and nonnecrotizing vasculitis being equally frequent. The SV were antineutrophil cytoplasmic antibody (ANCA)-associated in 22 patients (71%), and ANCA-negative in 9 cases (29%). Neither the type nor the clinical spectrum of the SV was predictive of MB positivity. None of the muscle symptoms (myalgias or biological rhabdomyolysis) were correlated with MB positivity. All the biopsies were performed uneventfully. CONCLUSION: The feasibility and positive predictive value of MB make it a valuable tool for ruling out a diagnosis of SV. Since no clinical signs could predict its positivity, MB should be considered in all suspected cases of SV. Unlike other biopsies, including kidney biopsy, MB had no prognostic value.

[Systemic vasculitis: study of 27 cases in Senegal]. / Vascularites systémiques: 27 observations au Sénégal.

Studies on vasculitis in black Africa are rare. The purpose of this report is to describe a retrospective study of systemic vasculitis managed in the internal medicine, ORL and cardiolology departments of the Aristide le Dantec University Hospital in Dakar, Senegal from 1995 to 2007. A series of 27 cases involving 7 men and 20 women with a mean age of 49 years was compiled. Primary vasculitis included Horton disease in 3 cases, Wegener disease in 2, Takayasu disease in 1, and Buerger disease in 1. Secondary vasculitis included mixed cryoglobulinemia with Gougerot Sjögren syndrome in 7 cases, primary Goujeröt syndrome in 4, rheumatoid arthritis in 3, nodosa periarteritis with hepatitis B in 2, SHARP syndrome in 1, and polymyositis in 1. The remaining two cases involved abdominal periaortitis including one associated with retrosperitoneal fibrosis and tuberculosis and the other with spondylarthropathy. Corticotherapy in combination with anticoagulants, immunosuppressive therapy, and surgery, when necessary, allowed effective management in 24 cases. The findings of this study show that systemic vasculitis can have numerous etiologies and indicate that secondary forms are the most common. Appropriate care modalities are needed to prevent severe outcome in Senegalese hospitals.

An approach to the diagnosis and management of systemic vasculitis.

The systemic vasculitides are a complex and often serious group of disorders which, while uncommon, require careful management in order to ensure optimal outcome. In most cases there is no known cause. Multi-system disease is likely to be fatal without judicious use of immunosuppression. A prompt diagnosis is necessary to preserve organ function. Comprehensive and repeated disease assessment is a necessary basis for planning therapy and modification of treatment protocols according to response. Therapies typically include glucocorticoids and, especially for small and medium vessel vasculitis, an effective immunosuppressive agent. Cyclophosphamide is currently the standard therapy for small vessel multi-system vasculitis, but other agents are now being evaluated in large randomized trials. Comorbidity is common in patients with vasculitis, including the cumulative effects of potentially toxic therapy. Long-term evaluation of patients is important in order to detect and manage relapses.