系统性硬化并发急性肾损伤的临床病理分析

Acute kidney injury of systemic sclerosis： scleroderma renal crisis and crescentic glomerulonephritis

目的 探讨系统性硬化（SSc）急性肾损伤（AKI）患者的临床和病理特点。方法 回顾性分析11例SSc急性肾损伤患者的临床资料，比较抗中性白细胞胞质抗体（ANCA）阳性和阴性SSc患者的肾脏损伤情况。结果 11例SSc急性肾损伤患者中，ANCA阴性9例，6例临床表现为硬皮病肾危象（SRC），其中4例肾活检示小叶间动脉和人球小动脉内皮细胞增生、肿胀，内膜黏液性水肿，内弹力纤维断裂，“洋葱皮样”改变和肾小球弥漫性缺血；无恶性高血压表现2例；急性肾小管坏死1例。MPO-ANCA阳性2例，无恶性高血压表现，1例肾活检示新月体肾炎。所有病例均予糖皮质激素治疗，8例患者接受环磷酰胺（CTX）治疗，9例患者接受血液透析治疗。SRC的6例患者都接受了大剂量的ACEI和（或）ARB治疗，其中4例于6个月内死亡，余ANCA阳性和阴性的患者中也各有1例死亡。结论 SSc患者AKI的临床表现多样，SRC、ANCA相关性小血管炎都可能导致SSc患者急性肾损伤发生，两者在临床、肾脏病理及治疗方面有明显的不同，但预后都不良。大剂量糖皮质激素有诱发SRC的可能，建议泼尼松用量〈15mg／d。

Objective To explore the clinicopathological characteristics of acute kidney injury （AKI） of systemic sclerosis （SSc）. Methods A retrospective study was performed on 11 SSc patients with AKI. The clinical data were analyzed and the patients were divided into antineutrophil cytoplasmic antibodies （ANCA） negative group （n=9） and ANCA positive （n=2） group. Results In the ANCA negative group, 2 cases were without malignant hypertension ,1 was acute tubular necrosis（ATN） caused by herbs, 6 were scleroderma renal crisis（SRC）, including 4 with renal biopsy, indicating hypertrophic arterial media, edema, thickened intima, onion-skin lesion in interlobular arteries and afferent arterioles, as well as ischemic lesion in glomeruli. In the MPO-ANCA positive group, 1 was crescentic glomerulonephritis. Malignant hypertension was not noticed. All patients were given steroid, 8 of them received CTX in addition. Nine patients received dialysis, and 8 cases progressed to permanent hemodialysis. Six cases with SRC were given high dose ACEI and / or ARB. Six patients resulted in early death. Conclusions Scleroderma renal crisis and ANCA associated vasculitis may cause AKI in SSc patients. Patients with positive ANCA differ from those with negative ANCA in terms of clinical manifestation, pathology and treatment. Survival and prognosis of SSc patient were bad. High dose corticosteroids increases the risk of scleroderma renal crisis, so it is thereby recommended that dose above 15 mg/ day should be avoided if possible.