病理表现为IgA肾病的变应性肉芽肿性血管炎1例报告及文献复习  

Churg-strausssyndrome manifested as IgA nephropathy:a case report and review of the literature

作　　者：于光[1] 张欢[1] 边琪[1] 傅鹏[1] 于建平[1] 崔若兰[1] 郭志勇[1] 

机构地区：[1]第二军医大学长海医院肾内科,上海200433

出　　处：《第二军医大学学报》2010年第11期1228-1231,共4页Academic Journal of Second Military Medical University

摘　　要：目的报道国内首例以IgA肾病为病理表现的变应性肉芽肿性血管炎1例。方法分析本例患者的临床病理资料及成立诊断的依据,并复习有关文献加以探讨。结果女性39岁患者,6年前因"肉眼血尿、蛋白尿"于我科行肾穿刺活检,病理诊断"IgA肾病,局灶节段性肾小球硬化";实验室检查偶见外周血嗜酸粒细胞比例增加,c-ANCA(抗中性粒细胞胞质抗体)/p-ANCA阳性各1次;给予足量泼尼松和环磷酰胺冲击治疗18个月,基本治愈。5年后,因血肌酐932μmol/L而行腹膜透析治疗。透析后一般状况无改善,先、后出现咳嗽、腹泻并伴有低热;经反复检查,外周血嗜酸粒细胞持续增高;p-AN-CA和MPO-ANCA阳性;CT示慢性鼻窦炎;既往有"皮疹"史;据此诊断为变应性肉芽肿性血管炎。给予人免疫球蛋白冲击治疗3d,泼尼松每日30mg口服。体温立即降至正常,一般状况显著好转,出院随访。结论 IgA肾病可能是CSS肾损伤的一种罕见的病理类型,在诊断时应予以考虑。Objective To report the first case of Churg-Strauss syndrome（CSS） manifested as IgA nephropathy in China.Methods The clinicopathological data for diagnosis of the patient were analyzed,and the related literatures were reviewed.Results The female patient aged 39 years old.Renal needle biopsy was performed 6 years ago because of gross hematuria and proteinuria,with the diagnosis being IgA nephropathy and focal segmental glomerulosclerosis.Laboratory examinations showed that the proportion of peripheral eosinophils was increased and c-ANCA/p-ANCA was positive once.Sufficient prednisone and cyclophosphamide were given for pulse therapy for 18 months,and the patient was clinically cured.One year ago,the patient received peritoneal dialysis because the serum creatinine reached 932 μmol/L.However,the general status of the patient was not improved after dialysis,and the patients developed cough,diarrhea and low-grade fever.Repeated blood test showed that the proportion of peripheral eosinophils was continuously increased.p-ANCA and MPO-ANCA were positive,and CT scan indicated chronic sinusitis.The patient had a history of＂rashes＂,therefore CSS was diagnosed.The patient was given pulse therapy with immunoglobulin treatment for 3 days and oral prednisone at a daily dose of 30 mg;the temperature of the patient decreased immediately to the normal level;and her general condition was improved and she was discharged.Conclusion IgA nephropathy was a rare pathological manifestation of CSS,which should be considered during diagnosis.

关 键 词：变应性肉芽肿性血管炎 IGA肾病 肺炎 抗中性白细胞胞质抗体 嗜酸粒细胞 

分 类 号：R543[医药卫生—心血管疾病]