他克莫司诱导治疗轻微系膜狼疮性肾炎合并狼疮足细胞病所致肾病综合征:一项基于病例的回顾。

Ibuki Kurihara, Chihiro Terai, Hiroki Yabe, Hitoshi Sugawara
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引用次数: 0

摘要

背景:由轻微系膜狼疮肾炎引起的肾病综合征被认为是罕见的。肾病综合征可由轻度系膜狼疮性肾炎伴弥漫性上皮足突消退和狼疮足细胞病引起。病例报告一名23岁的日本女性被诊断为混合性结缔组织病,入院前2周因体重增加和全身性水肿。她的脸颊有蝴蝶状红斑,蛋白尿,白细胞减少伴淋巴细胞减少和低白蛋白血症。她的抗核抗体呈阳性,特异性自身抗体仅对核糖核蛋白(RNP)抗原呈阳性。她被诊断为系统性红斑狼疮。肾活检显示肾小球轻微异常,免疫荧光显示外周有IgM和补体C3c沉积。电镜显示80%以上的毛细血管袢表面弥漫性足细胞足突消失,仅有少量内皮下沉积。因此,我们诊断轻微系膜狼疮肾炎伴狼疮足细胞病。在住院第4天,我们给予1000毫克/天的甲基强的松龙治疗3天,随后给予50毫克/天的强的松龙治疗,但蛋白尿持续存在。在第12天,我们给予他克莫司(3mg /天)。第17天蛋白尿改善后消失。泼尼松龙逐渐减量,3年后停用,他克莫司3mg /d继续使用。入院后4年未见急性发作。结论他克莫司对轻度系膜性狼疮性肾炎合并狼疮足细胞病有良好疗效。应该进行前瞻性和随机对照试验来证明他克莫司对这一适应症的疗效。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

Tacrolimus Induction Therapy for Nephrotic Syndrome Caused by Minimal Mesangial Lupus Nephritis with Lupus Podocytopathy: A Case-Based Review.

Tacrolimus Induction Therapy for Nephrotic Syndrome Caused by Minimal Mesangial Lupus Nephritis with Lupus Podocytopathy: A Case-Based Review.

Tacrolimus Induction Therapy for Nephrotic Syndrome Caused by Minimal Mesangial Lupus Nephritis with Lupus Podocytopathy: A Case-Based Review.

Tacrolimus Induction Therapy for Nephrotic Syndrome Caused by Minimal Mesangial Lupus Nephritis with Lupus Podocytopathy: A Case-Based Review.

BACKGROUND Nephrotic syndrome caused by minimal mesangial lupus nephritis is considered rare. Nephrotic syndrome can be caused by minimal mesangial lupus nephritis with diffuse epithelial foot-process effacement and lupus podocytopathy. CASE REPORT A 23-year-old Japanese woman diagnosed with mixed connective tissue disease was admitted because of weight gain and generalized edema for 2 weeks prior to admission. She had butterfly-shaped erythema on her cheeks, proteinuria, leukocytopenia with lymphocytopenia, and hypoalbuminemia. She was positive for antinuclear antibodies, and specific autoantibodies were only positive for the ribonucleoprotein (RNP) antigen. She was diagnosed with systemic lupus erythematosus. Renal biopsy showed minor glomerular abnormalities, and immunofluorescence revealed peripheral deposits of IgM and complement C3c. Electron microscopy revealed diffuse podocyte foot-process effacement of >80% of the capillary loop surfaces, with only a few subendothelial deposits. Consequently, we diagnosed minimal mesangial lupus nephritis with lupus podocytopathy. On hospital day 4, we administered 1000 mg/day of methylprednisolone for 3 days, followed by prednisolone 50 mg/day, but proteinuria persisted. On day 12, we administered tacrolimus (3 mg/day). Proteinuria improved and then disappeared on day 17. Prednisolone was gradually tapered and stopped after 3 years, although tacrolimus 3 mg/day was continued. No flare-up was observed 4 years after admission. CONCLUSIONS Tacrolimus showed good efficacy in this case of minimal mesangial lupus nephritis with lupus podocytopathy. Prospective and randomized controlled trials should be conducted to demonstrate the efficacy of tacrolimus for this indication.

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