系统性硬化症的肾脏损害

Clinicopathological studies of renal involvement in patients with systemic sclerosis

目的了解系统性硬化症肾脏损害的临床特点及病理特点。方法对我院９３例确诊的系统性硬化症（ＳＳｃ）患者进行回顾性临床分析，并对５例患者进行了肾组织病理学检查。结果有１９４％（１８例）患者有临床肾脏损害，肾功能衰竭发生率为５４％，本组病死率为１２９％，其中死于肾衰者占４１７％。４例患者和１例尸检的肾组织学显示ＳＳｃ的肾脏血管改变为多样性；４例显示有不同程度的小叶间动脉和弓动脉、入球动脉的受累，表现为血管内膜增厚，纤维组织呈“洋葱皮”样同心圆状排列，其中２例并无肾脏损害临床表现。另１例病程长达２２年的患者的肾脏病理表现主要为慢性肾小球肾炎。结论ＳＳｃ患者肾脏损害是一严重并发症，是其主要死亡原因。肾脏病理改变主要为肾脏血管病变，可发生在出现肾脏损害临床表现之前。对ＳＳｃ患者应密切加强随访。

bjective To assess the incidence of renal involvement in patients with systemic sclerosis (SSc) as well as its clinical and pathological changes. Methods The renal involvement was studied clinicopathologically in 93 patients who were compatible with the diagnosis of SSc restropectively. Results Eighteen patients (19.4%) were specified as having renal involvement by proteinuria, or renal hypertension, or elevated level of blood urea nitrogen (BUN) and/or serum creatinine (sCr). Renal failure was observed in 6 patients (54%) and 5 died. The mortality of the series was 129%. Histological studies were performed in 5 patients. The thickening of interlobular arteries demonstrated as omonskin like and intimal proliferation were the characteristic features of 4 patients whom also showed mild nonspecific glomerular changes. Two of the 4 were seen without abnormal clinical features, 1 with renal hypertension and 1 died of renal failure. The other patient with long duration of disease about 22 years showed chronic glomerulonephritis with nephrosclerosis. Conclusion The main pathological change of renal involvement in SSc was the intimal proliferation of the intrarenal arteries. It may be without clinical renal symptoms. Therefore SSc patients should be followed up clinically and received renal biopsy necessarily in order to discover early renal involvement and to insert rational therapy to improve its prognosis.