49岁女性，主要表现为乏力、贫血、肾功能不全，无蛋白尿、血尿，无咯血等肺部表现，血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，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，经甲泼尼龙和环磷酰胺治疗后，血ANCA转阴，重复肾活检肾小管间质急性病变亦明显减轻，肾功能明显改善，综合临床、实验室检查及组织学特点，最终诊断为ANCA相关性间质性肾炎。%A 49-year female was admitted because of fatigue, anemia, decreased renal function for six months. Serum pANCA and Elastase ANCA were positive. The renal biopsy was performed and histological examination demonstrated tubulointerstitial nephritis with some obsolescent, but otherwise near normal glomeruli. Immunofluorescence study indecated panci-immune deposits. The extensive investigations showed no signs of granulomatous inflammation or other vasculitic organ involvement. After immunosupressive treatment, serum ANCA was negative. Repeat biopsy showed significantly ameliorated interstitial inflammation. Renal function was obvionsly improved, the final diagnosis was suggested as ANCA associated tubulointerstitial nephritis.
展开▼
