Fatma YILDIRIM, Gülbin AYGENCEL, Abdurrahman TUFAN, Melda TÜRKOĞLU, İskender KARA

ANCA İlişkili Vaskülite Bağlı Gelişen Difüz Alveolar Hemorajide Plazmaferez Tedavisi: Olgu Sunumu ve Literatürün Gözden Geçirilmesi

Diffüz alveolar hemoraji (DAH), antinötrofil sitoplazmik antikor (ANCA) ilişkili vaskülitlerin en ciddi klinik durumlarından biridir. Burada perinükleer-ANCA ilişkili vaskülite bağlı akut renal yetmeliği ve DAH olan bir olgu sunulmuştur. Olgumuzda indüksiyon immünsupresif (kortikosteroid ve siklofosfamid) tedavilerine rağmen renal fonksiyonlarında bir düzelme olmamış ve DAH gelişmiştir. Kurtarma tedavisi olarak plazmaferez başlanmış ancak 5 seans sonrasında klinik tabloda düzelme olmadığı için kesilmiştir. Hasta takibinde muhtemel koroner tutuluma bağlı akut koroner sendrom nedeniyle kaybedilmiştir. Bu olgu ile birlikte progresif sistemik vasküliti olan olgularda plazmaferez tedavisinin kurtarıcı bir tedavi olarak kullanılabileceği; ancak sonuçların her zaman yüz güldürücü olmadığı sonucuna varılmıştır.

Plasmapheresis Therapy for ANCA Associated Vasculitis with Diffuse Alveolar Hemorrhage: A Case Report and Review of the Literature

Diffuse alveolar hemorrhage (DAH) is one of the most serious involvement of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. We report a case of a perinuclear-ANCA associated vasculitis with acute renal failure and DAH. The patient received induction immunosuppressive therapy (corticosteroid and cyclophosphamide), but patient's renal function didn't improve and then DAH occured. Plasmapheresis was started as rescue therapy. The DAH didn't resolve at the end of five procedures. Patient died of acute coronary syndrome due to probable coronary vascular involvement.We concluded that plasmapheresis rescue therapy can't get benefit in such a progressive systemic ANCA-associated vasculitis.

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