Anders H J, Wiebecke B, Haedecke C, Sanden S, Combe C, Schlöndorff D
Institute of Pathology, Ludwig-Maximilians-University, Munich, Germany.
Am J Kidney Dis. 1999 Apr;33(4):e3. doi: 10.1016/s0272-6386(99)70244-1.
Scleroderma renal crisis is characterized by intimal thickening of the afferent glomerular arterioles resulting in hypertension and fibrinoid necrosis of the capillary tuff. We report a 67-year-old man with long-standing systemic sclerosis who developed normotensive progressive renal failure, proteinuria, and a nephritic urinary sediment with serum myeloperoxidase-antineutrophil cytoplasmatic antibodies (MPO-ANCA). Renal biopsy showed pauci-immune crescentic glomerulonephritis but none of the typical vascular changes of scleroderma renal crisis. Because comparable cases have recently been reported from Japan, normotensive MPO-ANCA-positive crescentic glomerulonephritis may form an entity of progressive renal failure in scleroderma.
硬皮病肾危象的特征是肾小球入球小动脉内膜增厚，导致高血压和毛细血管襻的纤维素样坏死。我们报告一例67岁男性，患有长期系统性硬化症，出现血压正常的进行性肾衰竭、蛋白尿以及伴有血清髓过氧化物酶抗中性粒细胞胞浆抗体（MPO-ANCA）的肾炎性尿沉渣。肾活检显示寡免疫性新月体性肾小球肾炎，但没有硬皮病肾危象的典型血管改变。由于日本最近也报道了类似病例，血压正常的MPO-ANCA阳性新月体性肾小球肾炎可能是硬皮病中进行性肾衰竭的一种类型。
