Ishizu Akihiro, Fukaya Shinji, Tomaru Utano, Katsumata Kazuaki, Suzuki Akira, Umemoto Yuka, Furusaki Akira, Amasaki Yoshiharu
Faculty of Health Sciences, Hokkaido University, Sapporo, Hokkaido, Japan.
Ann Vasc Dis. 2012;5(4):458-61. doi: 10.3400/avd.cr.12.00078. Epub 2012 Nov 30.
This study presents a patient who died of acute renal failure (ARF) as a complication of scleroderma. The patient remained normotensive throughout the clinical course. Myeloperoxidase-anti-neutrophil cytoplasmic antibody was negative. Autopsy revealed fibrin thrombi in the glomerular capillaries and afferent arterioles, mesangiolysis, and double contour of the glomerular basement membrane. Contrarily, "onionskin lesions" of renal interlobular arteries, the histological hallmark of scleroderma renal crisis, were not discovered. These findings suggested that thrombotic microangiopathy (TMA) was the cause of ARF. Although the frequency is not high, close monitoring should be given to TMA in scleroderma because of possible mortality.
本研究报告了一名死于硬皮病并发症急性肾衰竭（ARF）的患者。该患者在整个临床过程中血压正常。髓过氧化物酶抗中性粒细胞胞浆抗体为阴性。尸检发现肾小球毛细血管和入球小动脉中有纤维蛋白血栓、系膜溶解以及肾小球基底膜双轨征。相反，未发现肾小叶间动脉的“洋葱皮样病变”，而这是硬皮病肾危象的组织学特征。这些发现提示血栓性微血管病（TMA）是ARF的病因。尽管发生率不高，但由于可能导致死亡，对于硬皮病患者中的TMA应密切监测。
