A 49-year female was presented with fatigue, hypopotassemia, anemia, and renal dysfunction for half a year. The laboratary investigation showed tubulointerstitial injury without proteinuria and hemoturia. Serum pANCA and Elastase ANCA were positive. The renal histological examination demonstrated tubulointerstitial nephritis with peritubular capillaritis, tubulitis and some obsolescent, but otherwise near normal glomeruli. Immunoflurescence study indicated pauci-immune deposits. After immunosuppressive treatment, serum ANCA turned negative and renal function was remarkably improved. Repeat biopsy showed that the interstitial inflammation was significantly ameliorated. Finally, ANCA associated tubulointerstitial nephritis was diagnosed.%49岁女性,以乏力、低钾起病,近半年出现贫血,肾功能不全,无血尿,蛋白尿,无咯血等肺部表现,肾小管间质损害明显,血清核周型抗中性粒细胞胞质抗体(pANCA)阳性,弹性蛋白酶ANCA阳性.肾活检组织学表现为间质性肾炎,可见大量管周毛细血管炎,肾小管炎,伴肾小球废弃,非废弃肾小球病变轻微,免疫病理示寡免疫复合物沉积.甲泼尼龙和环磷酰胺冲击治疗后,血清ANCA转阴,随之肾功能改善,重复肾活检肾小管间质急性病变也明显减轻.结合临床、实验室检查,对免疫抑制治疗反应及两次肾活检组织学特点,最终诊断为ANCA相关性间质肾炎.
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