Müller-Höcker J, Schmid H, Weiss M, Dendorfer U, Braun G S
Pathologische Institut and medizinische Klinik-Poliklinik, Ludwig-Maximilians-Universitãt, Munich, Germany.
Hum Pathol. 2003 Mar;34(3):285-9. doi: 10.1053/hupa.2003.36.
A 46-year-old female patient with Sjögren's syndrome, hypertension, and stable chronic renal insufficiency (creatinine [CR], 1.9 to 2.1 mg/dL) had a progressive worsening of renal function (CR, 5.0 mg/dL) after 11 months of chloroquine therapy (155 mg/day; cumulative dose of approximately 51 g). Light microscopy revealed nonspecific angionephrosclerosis. Electron microscopy showed accumulations of lamellated myelinoid material and occasionally also of curvilinear bodies, especially in the glomerular podocytes and to a lesser extent in vascular myothelial and endothelial cells. In the tubular system, mainly protein droplets were stored. Activity of alpha-galactosidase A was normal in isolated leukocytes (56 nmol/mg; range, 33.2 to 109 nmol/mg), ruling out Fabry's disease. Clinical, morphological, and biochemical findings were consistent with chloroquine-associated deterioration of renal function that improved considerably after discontinuation of chloroquine treatment. Adverse effects of chloroquine may aggravate preexisting renal disease. Electron microscopy is a worthwhile tool for establishing the correct diagnosis.
一名46岁患有干燥综合征、高血压且慢性肾功能不全稳定（肌酐[CR]，1.9至2.1mg/dL）的女性患者，在接受氯喹治疗11个月（155mg/天；累积剂量约51g）后，肾功能逐渐恶化（CR，5.0mg/dL）。光镜检查显示非特异性血管性肾硬化。电镜检查发现有层状髓鞘样物质积聚，偶尔也有曲线体积聚，尤其在肾小球足细胞中，在血管肌内皮细胞和内皮细胞中程度较轻。在肾小管系统中，主要储存的是蛋白滴。分离的白细胞中α-半乳糖苷酶A活性正常（56nmol/mg；范围为33.2至109nmol/mg），排除了法布里病。临床、形态学和生化检查结果与氯喹相关的肾功能恶化一致，在停用氯喹治疗后肾功能有显著改善。氯喹的不良反应可能会加重已有的肾脏疾病。电镜检查是确立正确诊断的一项有价值的工具。
