我が国の急速進行性糸球体腎炎の治療におけるアフェレシス療法の位置づけ(<特集>腎・血液領域の注目すべき疾患-アフェレシス療法のエビデンスとガイドライン-)

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A national survey concerning rapidly progressive glomerulonephritis (RPGN) was conducted in Japan between 1989 and 2004 and resulted in the registration of 1,342 patients with RPGN. Among the documented patients, the most frequent primary disease was primary pauciimmune crescentic glomerulonephritis, and the second most frequent was microscopic polyangitis. Overall, more than 60% of the patients had MPO-ANCA, 5% of the patients had anti-GBM antibody, and only 3.3% of the patients had PR 3-ANCA. We found that efficacy of apheresis was not observed among anti-GBM antibody mediated RPGN due to late referral to each institution. Eighty-three patients received apheresis therapy with various combinations of immunosuppressive regimens in MPO-ANCA-associated RPGN patients. They had higher serum creatinine, higher CRP, and a higher frequency of complicated pulmonary involvements as compared to the controls without apheresis therapy. In dialysis-dependent patients, no additional benefit from apheresis therapy was observed. Only pulmonary renal syndrome patients with CRP>6mg/dl at presentation showed a slightly better prognosis (patient survival with apheresis, 66.7%; without apheresis, 56.7%). Furthermore, a rapid MPO-ANCA titer reduction was observed in patients treated with apheresis. Patients with MPO-ANCA-associated RPGN were older, and had more chronic and sclerotic lesions than patients with PR 3-ANCA-associated RPGN. Based on these findings, we suggest that a lower dose of immunosuppressant should be considered in order to avoid opportunistic infection. Nevertheless, in patients with an aggressive form of RPGN with rapid deterioration of renal function like the PR 3-ANCA-associated RPGN, or pulmonary renal syndrome complicated severe inflammation, or relapses with high MPO-ANCA titer, we conclude that apheresis therapy should be considered.
日本アフェレシス学会の論文
2006-02-28

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