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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 a case of allergic granulomatous vasculitis, the first case of IgA nephropathy in China. Methods To analyze the clinical and pathological data of patients and the basis for the establishment of diagnosis, and to review the literature to be explored. Results Female 39 years old, 6 years ago due to “gross hematuria, proteinuria ” in our department of renal biopsy, pathological diagnosis “IgA nephropathy, focal segmental glomerulosclerosis ”; Laboratory tests even See the increase in the proportion of peripheral blood eosinophils, c-ANCA (anti-neutrophil cytoplasmic antibody) / p-ANCA positive one; given sufficient prednisone and cyclophosphamide shock treatment for 18 months, the basic cure . 5 years later, due to serum creatinine 932μmol / L peritoneal dialysis treatment. After dialysis, there was no improvement in general condition. First, cough, diarrhea accompanied with low fever were observed. Peripheral blood eosinophilia continued to be elevated after repeated examination. Positive p-AN-CA and MPO-ANCA showed chronic sinusitis with CT. Have “rash ” history; accordingly diagnosed as allergic granulomatous vasculitis. Give human immunoglobulin shock treatment 3d, prednisone 30mg orally daily. Body temperature immediately dropped to normal, the general condition significantly improved, discharged from hospital. Conclusion IgA nephropathy may be a rare pathological type of CSS renal injury, which should be considered in the diagnosis.