MPO-ANCA関連血管炎に対する白血球系細胞除去療法(<特集>血球成分除去療法の新しい展開)

概要

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Anti-neutrophil cytoplasmic antibody (ANCA)-associated systemic vasculitis (AASV) is characterized by rapidly progressive glomerulonephritis (RPGN) and lung hemorrhage. The prognosis of severe AASV with multi-organ involvement has improved with combined therapy of cyclophosphamide and high-dose corticosteroids. However, the most common cause of death in patients with RPGN is infection. Therefore, additional therapy by which the total dose of drugs can be reduced is desirable. The presence of lung hemorrhage is associated with poor outcome. Therefore, prompt institution of supportive measures and therapy is essential for survival. We reviewed the records of 27 patients with MPO-ANCA-associated glomerulonephritis and 5 patients with lung hemorrhage due to MPO-ANCA-associated vasculitis who were all treated by cytapheresis. Renal function improved with oral corticosteroid and cytapheresis in 23 of the 27 patients, and severe infection occurred in 1 of the 27 patients. Lung hemorrhage ameliorated in all 5 patients. Cytapheresis could become an optional therapy for the treatment of MPO-ANCA-associated vasculitis. Further studies are necessary to fully evaluate the efficacy of cytapheresis in patients with MPO-ANCA-associated vasculitis.
日本アフェレシス学会の論文
2009-02-28