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他克莫司改善快速进展性狼疮肾炎相关 MPO-ANCA。

Improvement of rapidly progressive lupus nephritis associated MPO-ANCA with tacrolimus.

机构信息

Department of Internal Medicine and Rheumatology, Juntendo University School of Medicine, 2-1-1 Hongo, Bunkyo-ku, Tokyo, Japan.

出版信息

Mod Rheumatol. 2010 Jun;20(3):291-4. doi: 10.1007/s10165-009-0266-x. Epub 2010 Jan 15.

Abstract

A 43-year-old Japanese woman with systemic lupus erythematosus (SLE) developed rapidly progressive renal failure and nephritic syndrome with a high titer of myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA). Methylprednisolone pulse therapy did not suppress the progression of renal failure. Intravenous cyclophosphamide pulse therapy and administration of azathioprine was abandoned due to adverse effects. Tacrolimus was employed as an alternative immunosuppressive therapy and was well tolerated, effectively preventing renal failure. Oral prednisolone dosage was successfully tapered without recurrence, along with decreasing titer of MPO-ANCA. Renal biopsy showed diffuse proliferative lupus nephritis (International Society of Nephrology/Renal Pathology Society class IV-G A/C) with crescent formation. These findings indicate that in addition to lupus nephritis, which usually results from deposition of circulating or locally formed immune complexes, MPO-ANCA may be involved in the pathogenesis of crescentic glomerulonephritis. Furthermore, we propose that tacrolimus is an effective immunosuppressant for MPO-ANCA-related renal crisis in diffuse proliferative lupus nephritis.

摘要

一位 43 岁的日本女性患有系统性红斑狼疮 (SLE),出现了快速进展性肾衰竭和伴有髓过氧化物酶-抗中性粒细胞胞质抗体 (MPO-ANCA) 高滴度的肾炎综合征。甲泼尼龙脉冲治疗未能抑制肾衰竭的进展。由于不良反应,放弃了静脉环磷酰胺脉冲治疗和硫唑嘌呤治疗。环孢素 A 作为替代免疫抑制剂治疗,效果良好,能有效预防肾衰竭。口服泼尼松剂量成功逐渐减少,没有复发,MPO-ANCA 滴度也下降。肾活检显示弥漫性增生性狼疮性肾炎 (国际肾脏病学会/肾脏病理学会分类 IV-G A/C) 伴有新月体形成。这些发现表明,除了通常由循环或局部形成的免疫复合物沉积引起的狼疮肾炎外,MPO-ANCA 可能参与新月体性肾小球肾炎的发病机制。此外,我们提出环孢素 A 是治疗弥漫性增生性狼疮性肾炎中 MPO-ANCA 相关肾危象的有效免疫抑制剂。

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