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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 clinical diagnosis and treatment of mixed connective tissue disease (MCTD) associated with anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis. METHODS: A 35-year-old Asian woman admitted to hospital with two-hand Raynaud’s phenomenon, myalgia, arthralgia and fatigue. The patient was diagnosed with MCTD in the clinic eight months before admission. Raynaud’s phenomenon, myalgia, arthralgia and high serum U1RNP antibody in patients met the Alarcon-Segovia classification criteria of MCTD. After admission examination found that patients with serum myeloperoxidase (MPO) anti-neutrophil cytoplasmic antibody positive, 24 h urinary protein increased significantly. Chest CT showed interstitial lung disease (ILD) features. Subsequent renal biopsy prompted histological fibroblast / cell crescent glomerulonephritis, direct fluorescence staining showed moderate IgM positive. RESULTS: Patients were given 500 mg / d methylprednisolone for 3 days immediately after obtaining the renal histopathological examination and then changed to oral prednisone and 0.8 g cyclophosphamide monthly for 12 months. After the above treatment, patients with serum creatinine remained normal, a significant decrease in the amount of daily urinary protein. CONCLUSION: Glucocorticoid combined with cyclophosphamide can effectively treat MPO-ANCA-associated glomerulonephritis with MCTD.