EFFICACY OF IMMUNOSUPPRESSIVE AND ANTICOAGULANT THERAPY FOR THE GRANULOMATOSIS PATIENT WITH WITH POLYANGIITIS AND CRYOFIBRINOGENEMIA


T.P. Shevtsova (1), P.I. Novikov (2), I.O. Smitienko (3), A.D. Meshkov (4), S.V. Moiseev (4)

(1) Department of Internal Diseases FFM MSU n.a. M.V. Lomonosov, Moscow; (2) Rheumatological unit UCH № 3 SBEI HPE "First MSMU n.a. I.M. Sechenov" of RMPH, Moscow; (3) Department of Internal Diseases, Cardiology and Clinical Pharmacology FATMP PFUR, Moscow; (4) Department of Internal, Occupational Diseases and Pulmonology MPF SBEI HPE "First MSMU n.a. I.M. Sechenov" of RMPH, Moscow
Granulomatosis with polyangiitis (GPA) (Wegener’s granulomatosis) - one of the ANCA-associated vasculitides, which usually occurs with severe visceral lesions determining the prognosis for most patients. The article presents the clinical observation of the 29-years-old patient, which was diagnosed with GPA with severe necrotizing defects and pulmonary disorders after two years of follow-up. In September 2006, due to the spreading necrosis on the lower and upper extremities and ascending gangrene, amputation of both legs - left above the knee, the right above upper third of the tibia – and amputation of distal phalanx of the fingers were performed. In 2010, cryofibrinigenemia without cryoglobulinemia was revealed for the first time. This clinical observation shows how the presence of cryofibrinogenemia may change the course of ANCA-associated vasculitides. High clinical and laboratory disease activity led to massive immunosuppressive therapy, which provided a positive trend.

About the Autors


T.P. Shevtsova – Teaching Assistant at the Department of Internal Diseases FFM MSU n.a. M.V. Lomonosov; e-mail: TPShevcova@gmail.com
P.I. Novikov – Head of the Rheumatological unit UCH № 3 SBEI HPE "First MSMU n.a. I.M. Sechenov" of RMPH; e-mail: novikov-pavel@mail.ru
I.O.Smitienko – PhD in Medical Sciences, Assistant Professor at the Department of Internal Diseases, Cardiology and Clinical Pharmacology FATMP PFUR
A.D.Meshkov – Teaching Assistant at the Department of Internal, Occupational Diseases and Pulmonology MPF SBEI HPE "First MSMU n.a. I.M. Sechenov" of RMPH; e-mail: alexeymeshkov@mail.ru
S.V.Moiseev – MD, Prof. at the Department of Internal, Occupational Diseases and Pulmonology MPF SBEI HPE "First MSMU n.a. I.M. Sechenov" of RMPH; e-mail: clinpharm@mtu-net.ru


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