Division of General Medicine, Department of Comprehensive Medicine 1, Saitama Medical Center, Jichi Medical University, Saitama, Japan.
Division of Rheumatology, Department of Comprehensive Medicine 1, Saitama Medical Center, Jichi Medical University, Saitama, Japan.
Am J Case Rep. 2022 Nov 7;23:e937201. doi: 10.12659/AJCR.937201.
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.
由微小病变性狼疮肾炎引起的肾病综合征被认为较为罕见。肾病综合征可由弥漫性上皮足突消失和狼疮性足细胞病引起的微小病变性狼疮肾炎引起。
一名 23 岁的日本女性,患有混合性结缔组织病,因入院前 2 周体重增加和全身水肿而入院。她面颊有蝶形红斑,蛋白尿,白细胞减少伴淋巴细胞减少,低白蛋白血症。她抗核抗体阳性,特异性自身抗体仅核糖核蛋白(RNP)抗原阳性。她被诊断为系统性红斑狼疮。肾活检显示肾小球有轻微病变,免疫荧光显示免疫球蛋白 M 和补体 C3c 呈周围沉积。电子显微镜显示弥漫性足细胞足突消失>80%的毛细血管袢表面,仅少数内皮下沉积。因此,我们诊断为微小病变性狼疮肾炎合并狼疮性足细胞病。入院第 4 天,我们给予 1000mg/天甲基强的松龙治疗 3 天,然后给予泼尼松 50mg/天,但蛋白尿持续存在。入院第 12 天,我们给予他克莫司(3mg/天)。入院第 17 天蛋白尿改善并消失。泼尼松逐渐减量并在 3 年后停药,尽管继续给予他克莫司 3mg/天。入院 4 年后未观察到病情复发。
在本例微小病变性狼疮肾炎合并狼疮性足细胞病中,他克莫司显示出良好的疗效。应进行前瞻性和随机对照试验,以证明他克莫司在该适应证中的疗效。