1例混合性结缔组织病合并ANCA相关肾小球肾炎患者(英文)  

ANCA associated glomerulonephritis in a patient with mixed connective tissue disease

作　　者：孙怡宁[1] 何岚[1] 吕晓虹[1] 莫凌菲[1] 张竞[1] 

机构地区：[1]西安交通大学医学院第一附属医院风湿科,西安710061

出　　处：《中南大学学报（医学版）》2014年第2期209-314,共6页Journal of Central South University ：Medical Science

摘　　要：目的:探讨临床上十分罕见的混合性结缔组织病(mixed connective tissue disease,MCTD)同时合并抗中性粒细胞胞浆抗体(anti-neutrophil cytoplasmic antibodies,ANCA)相关血管炎的诊断和治疗。方法:1名35岁的亚裔女性,因双手雷诺现象、肌痛、关节痛和乏力入院。在入院前8个月该患者在门诊诊断为MCTD。患者的雷诺现象、肌痛、关节痛和血清高滴度抗U1核糖核蛋白(U1 ribonucleoprotein,U1RNP)抗体符合MCTD的Alarcon-Segovia分类标准。入院后的检查发现患者血清髓过氧化物酶(myeloperoxidase,MPO)型抗中性粒细胞胞浆抗体阳性、24 h尿蛋白定量显著升高。胸部CT显示间质性肺病(interstitial lung disease,ILD)特征。随后的肾穿刺活检提示组织学上表现为纤维细胞/细胞新月体型肾小球肾炎,直接荧光染色显示中度IgM阳性。结果:获得肾组织病理学检查结果后立即给予患者500 mg/d甲泼尼龙静脉冲击治疗3 d,随后改为口服泼尼松和每月0.8 g环磷酰胺静脉注射,持续12个月。经上述治疗,患者血清肌酐维持正常,每日尿蛋白量显著下降。结论:糖皮质激素联合环磷酰胺可以有效治疗MCTD合并的MPO-ANCA相关肾小球肾炎。Objective: To investigate the diagnosis and treatment of mixed connective tissue disease(MCTD) and myeloperoxidase-antineutrophil cytoplasmic antibody(MPO-ANCA) associated vasculitis, which is a rare clinical entity in medical practice. Methods: A 35-year-old female of Asian origin was admit ed to our hospital due to complaints of Raynaud's phenomenon, myalgia, arthralgia and fatigue. h e patient was diagnosed as MCTD in the out-patient department 8 months prior to admission based on Alarcon-Segovia classii cation criteria of Raynaud's phenomenon, myalgia, arthralgia and a high anti-U1 ribonucleoprotein antibody level. Interstitial lung disease was determined by chest computed tomography. Renal biopsy was performed because of marked proteinuria on 24 h urine collection. Histopathological examination revealed glomerulonephritis with fibrocellular/cellular crescents, in which moderate staining of IgM was shown by direct immunol uorescence. She was tested positive for myeloperoxidase antineutrophil cytoplasmic antibody. Results: High dose of methylprednisolone(500 mg/d for 3 days) was started intravenously when the results of renal biopsy were obtained. Oral prednisone and intravenous cyclophosphamide therapy(0.8 g/month) were continued for 12 months. Daily urinary protein loss decreased dramatically and serum creatinine was maintained at a normal level. Conclusion: Corticosteroids and cyclophosphamide are ef ective in the treatment of MPO-ANCA associated glomerulonephritis in MCTD.

关 键 词：混合性结缔组织病 髓过氧化物酶 抗中性粒细胞胞浆抗体 肾小球肾炎 血管炎 

分 类 号：R692.31[医药卫生—泌尿科学]