Akioka Yuko, Hattori Motoshi, Kawaguchi Hiroshi, Ito Katsumi
Department of Pediatric Nephrology, Kidney Center, Tokyo Women's Medical University School of Medicine, Tokyo, Japan.
Ther Apher Dial. 2004 Jun;8(3):223-6. doi: 10.1111/j.1526-0968.2004.00150.x.
We report the case of a 4.5-year-old girl with microscopic polyangiitis (MPA) manifesting antineutrophil cytoplasmic autoantibody (ANCA)-positive necrotizing crescentic glomerulonephritis and pulmonary hemorrhage. She was initially on induction therapy with corticosteroids and azathioprine. Plasma exchange (PE) combined with immunosuppressants was used to treat an episode of recurrent pulmonary hemorrhage, and achieved remission. At 9.8 years of age her kidney disease relapsed, associated with renal dysfunction and increased proteinuria. To minimize the toxic effects of immunosuppressants, she was treated with PE again, and her renal dysfunction resolved. Plasma exchange was effective in reducing the risk of death and preserving long-term renal function without the severe adverse effects of immunosuppressants. Our preliminary results indicate that PE is likely to be a treatment option for children in acute phase of ANCA-associated MPA, who should be protected from the toxic effects of immunosuppressants.
我们报告了一例4.5岁女童患显微镜下多血管炎（MPA）的病例，该病例表现为抗中性粒细胞胞浆抗体（ANCA）阳性的坏死性新月体性肾小球肾炎和肺出血。她最初接受了皮质类固醇和硫唑嘌呤的诱导治疗。血浆置换（PE）联合免疫抑制剂用于治疗复发性肺出血发作，并实现了缓解。9.8岁时，她的肾病复发，伴有肾功能不全和蛋白尿增加。为尽量减少免疫抑制剂的毒性作用，她再次接受了PE治疗，肾功能不全得到缓解。血浆置换有效地降低了死亡风险并保留了长期肾功能，且无免疫抑制剂的严重不良反应。我们的初步结果表明，对于处于ANCA相关性MPA急性期的儿童，血浆置换可能是一种治疗选择，这些儿童应免受免疫抑制剂的毒性影响。
