Plasma Exchange in a Patient with Antineutrophil Cytoplasmic Antibody-associated Vasculitis / 대한수혈학회지

ABSTRACT

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis is a systemic autoimmune disease with a poor prognosis in untreated patients, and combination therapy with steroidsand cyclophosphamide is the current standard treatment. Treatment with immunosuppressive agents results in high remission rates; however, there is a considerable morbidity related to the immunosuppressive state, such as life-threatening infections. To reduce serious immunosuppressant-related adverse effects, potentially promising treatment options, including plasma exchange, cytapheresis, administration of intravenous immunoglobulin and TNF-alpha blocking agents have been developed. We performed plasma exchange with steroid pulse therapy for a 52 year-old female patient with pulmonary hemorrhagewith ANCA-associated crescentic glomerulonephritis. After combination therapy, the pulmonary hemorrhage and renal function recovered and myeloperoxidase (MPO)-ANCA decreased.