Effektivnost' immunosupressivnoy i antikoagulyantnoy terapii u bol'noy granulematozom s poliangiitom i kriofibrinogenemiey


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Resumo

Granulomatosis with polyangiitis (Wegener's granulomatosis) usually occurs with severe visceral involvement determining the prognosis for most patients. The article presents the clinical observation of the 29-yearsold patient, which although active immunosuppressive therapy improve visceral lesions, had severe necrotizing defects, ascending gangrene, amputation of both legs. Subsequently cryofibrinigenemia was revealed, und treatment with anticoagulants was effective - skin lesions were disappeared. This clinical observation shows how the presence of cryofibrinogenemia may change the course of ANCA-associated vasculitides.

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