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


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Abstract

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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  1. Новиков П.И., Семенкова Е.Н., Моисеев С.В. Современная номенклатура системных васкулитов. Клиническая фармакология и терапия. 2013; 1: 70-4.
  2. Новиков П.И., Моисеев С.В., Кузнецова Е.И., Семенкова Е.Н., Мухин Н.А. Изменения течения заболевания и прогноза гранулематоза с полиангиитом (Вегенера): результаты 40-летнего наблюдения. Клиническая фармакология и терапия. 2014; 1: 32-7
  3. Моисеев С.В., Новиков П.И., Мешков А.Д., Иваницкий Л.В. АНЦА-ассоциированные васкулиты: спорные вопросы классификации, диагностики и оценки активности и современные подходы к лечению. Клиническая фармакология и терапия. 2014; 1: 44-50.
  4. Новиков П.И., Моисеев С.В., Буланов Н.М., Макаров Е.А. Современные подходы к терапии АНЦА-ассоциированных системных васкулитов. Клиническая нефрология. 2014; 1: 42-9.
  5. Allenbach Y., Seror R., Pagnoux C., et al. High frequency of venous thromboembolic events in Churg-Strauss syndrome, Wegener's granulomatosis and microscopic polyangiitis but not polyarteritis nodosa: a systematic retrospective study on 1130 patients. Ann. Rheum. Dis. 2009; 68(4): 564-67.
  6. Soyfoo M.S., Goubella A., Cogan E., et al. clinical significance of cryofibrinogenemia: possible pathophysiological link with Raynaud's phenomenon. J. Rheumatol. 2011; 39: 119-24.
  7. Sebastian J.K., Voetsch B., Stone J.H., et al. The frequency of anticardiolipin antibodies and genetic mutations associated with hypercoagulability among patients with Wegener's granulomatosis with and without history of a thrombotic event. J. Rheumatol. 2007; 34: 2446-50.
  8. Lamprecht P., de Groot K., Schnabel A., et al. Anticardiolipin antibodies and antibodies to beta(2)-glycoprotein I in patients with Wegener's granulomatosis. Rheumatology. 2000; 39: 568-70.
  9. Merkel P.A., Chang Y., Pierangeli S.S., et al. The prevalence and clinical association of anticar-diolipin antibodies in a large inception cohort of patients with connective tissue diseases. Am. J. Med. 1996; 101: 576-83.
  10. Korst D.R., Kratochvil C.H. Cryofibrinogen in a case of lung neoplasm associated with thrombophlebitis migrans. Blood. 1955; 10: 945-53.
  11. Vermeersch P., Gijbels K., Marien G., et al. A Critical Appraisal of Current Practice in the Detection, Analysis, and Reporting of Cryoglobulins. Clin. Chem. 2008; 54: 1: 39-43.
  12. Smith R.T., Von Korff R.W. A heparin-precipitable fraction of human plasma. I. Isolation and characterization of the fraction. J. Clin. Investig. 1957; 36: 596-604.
  13. Smith R.T. A heparin-precipitable fraction of human plasma. Occurrence and significance of the fraction in normal individuals and in various disease states. J. Clin. Investig. 1957; 36: 605-16.
  14. Martin M., Pourrat J. Cryofibrinogenaemia. J. Clin. Rheumatol. 2013; 19(3): 142-48.
  15. Blain H., Cacoub P., Musset L., et al. Cryofibrinogenaemia: a study of 49 patients. Clin. Exp. Immunol. 2000; 120: 253-60.
  16. Saadoun D., Elalamy I., Ghillani-Dalbin P., et al. Cryofibrinogenemia: new insights into clinical and pathogenic features. Am. J. Med. 2009; 122: 1128-35.
  17. Amdo T.D., Welker J.A. An approach to the diagnosis and treatment of cryofibrinogenemia. Am. J. Med. 2004; 116: 332-37.
  18. Saadoun D., Musset L., Cacoub P. Cryofibrinogenemia. Rev. Med. Interne Fondee. 2010; 32: 287-91.
  19. Belizna C., Loufrani L., Subra J.F., et al. A 5-year prospective follow-up study in essential cryo fibrinogenemia patients. Autoimmune Rev. 2011; 10: 559-62.
  20. Belizna C.C., Tron F., Joly P., et al. Outcome of essential cryofibrinogenaemia in a series of 61 patients. Rheumatology. 2008; 47: 205-7.
  21. Nash J.W., Ross P. Jr, Neil Crowson A., et al. The histopathologic spectrum of cryofibrinogenemia in four anatomic sites. Skin, lung, muscle, and kidney. Am. J. Clin. Pathol. 2003; 119: 114-22.
  22. Terrier B., Izzedine H., Musset L., et al. Prevalence and clinical significance of cryofibrinogenaemia in patients with renal disorders. Nephrol. Dial. Transplant. 2011; 26: 3577-81.
  23. Zlotnick A., Shahin W., Rachmilewitz E.A. Studies in cryofibrinogenemia. Acta Haematol. 1969; 42: 8-17.
  24. McKee P.A., Bird R.M. Incidence and significance of cryofibrinogenemia. J. Lab. Clin. Med. 1962; 61: 203-10.
  25. Sawada K., Takahashi R., Saniabadi A.R., et al. Elevated plasma cryofibrinogen in patients with active inflammatory bowel disease is morbigenous. World J. Gastroenterol. 2006; 12: 1621-25.
  26. Kuipers J.G., Kellett J., May D. Low levels of cryofibrinogenaemia and peripheral circulatory dysfunction. Irish Med. J. 1991; 84: 68-9.
  27. Nagy J., Ambrus M., Paal M., et al. Cryoglobulinaemia and cryofibrinogenaemia in IgA nephropathy: a follow-up study. Nephron. 1987; 46: 337-42.
  28. Stathakis N.E., Karamanolis D., Koukoulis G., et al. Characterization of cryofibrinogen isolated from patients plasma. Haemostasis. 1981; 10: 195-202.
  29. Smith S.B., Arkin C. Cryofibrinogenemia: incidence, clinical correlations, and a review of the literature. Am. J. Clin. Pathol. 1972; 58: 524 -30.
  30. Rachmilewitz E.A., Sacks M.I., Zlotnick A. Essential cryofibrinogenemia. Clinical, pathological and immunological studies. Isr. J. Med. Sci. 1970; 6: 32-43.
  31. Jantunen E., Soppi E., Neittaanmaki H., et al. Essential cryofibrinogenaemia, leukocytoclastic vasculitis and chronic purpura. J. Intern. Med. 1993; 234: 331-33.
  32. Kirsner R.S., Eaglstein W.H., Katz M.H., et al. Stanozolol causes rapid pain relief and healing of cutaneous ulcers caused by cryofibrinogenemia. J. Am. Acad. Dermatol. 1993; 28: 71-4.
  33. Revenga F., Aguilar C., Gonzalez R., et al. Cryofibrinogenaemia with a good response to stanozolol. Clin. Exp. Dermatol. 2000; 25: 621-23.
  34. Williamson A.E., Cone L.A., Huard G.S. 2nd. Spontaneous necrosis of the skin associated with cryofibrinogenemia, cryoglobulinemia, and homocystinuria. Ann. Vasc. Surg. 1996; 10: 365-69.
  35. Helfman T., Falanga V. Stanozolol as a novel therapeutic agent in dermatology. J. Am. Acad. Dermatol. 1995; 33: 254-58.
  36. Euler H.H., Zeuner R.A., Beress R., et al. Monoclonal cryo-antifibrinogenemia. Arthritis Rheum. 1996; 39: 1066-69.
  37. Copeman P.W. Cryofibrinogenaemia and skin ulcers: treatment with plasmapheresis. Br. J. Dermatol. 1979; 101(Suppl 17): 57-8.
  38. Sankarasubbaiyan S., Scott G., Holley J.L. Cryofibrinogenemia: an addition to the differential diagnosis of calciphylaxis in end-stage renal disease. Am. J. Kidney Dis. 1998; 32: 494-98.
  39. Browning C.E., Callen J.P. Warfarin therapy for livedoid vasculopathy associated with cryofibrinogenemia and hyperhomocysteinemia. Arch. Dermatol. 2006; 142: 75-8.
  40. Beightler E., Diven D.G., Sanchez R.L., et al. Thrombotic vasculopathy associated with cryofibrinogenemia. J. Am. Acad. Dermatol. 1991; 24: 342-45.

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