系统性硬化症肾危象5例临床特点分析及文献复习

目的 分析系统性硬化症肾危象的临床特点.方法 观察近年我院收治5 例系统性硬化症肾危象患者的临床表现,实验室指标,肾脏病理表现及治疗转归,总结疾病的特点并文献复习.结果 5 例中女性4 例,男性1 例,平均年龄51 岁,平均病程22.2 月.临床表现有皮肤紧硬、关节肿痛、雷诺现象等;1 例患者有肌痛肌无力.1 例有肺间质纤维化,2 例有肺动脉高压,1 例有肺动脉高压和肺间质纤维化.5 例均有高血压和肾功能不全.4 例经肾穿刺活检诊断,1 例根据临床症状诊断.5 例均为弥漫性皮肤型系统性硬化症,其中3 例经治疗后病情好转,2 例死亡.结论 系统性硬化症肾危象进展较快,病情凶险,应及早判断.

Clinical features of scleroderma renal crisis: A report of 5 cases and review of the literature

Objective To analyze the clinical features of scleroderma renal crisis(SRC).Methods Clinical manifestations,laboratory data,pathological findings and treatment outcome of 5 patients with SRC were retrospectively analyzed with the related literature reviewed.Results The 5 patients(4 females and 1 male) was with the average age of 51 years and with an average disease course of 22.2 months.Their main clinical manifestations were thickening and tethering of skin,joint swelling pain and Raynaud phenomenon.Of the 5 patients,1 suffered from muscle pain and weakness,1 pulmonary interstitial fibrosis,2 pulmonary hypertension and 1 pulmonary interstitial fibrosis and pulmonary hypertension.The 5 patients suffered from hypertension and renal insufficiency.Of these patients,4 were diagnosed by renal biopsy and 1 was diagnosed according to his clinical symptoms.Of the 5 patients with diffuse cutaneous scleroderma,3 improved after treatment and 2 died.Conclusion SRC is a severe complication of systemic sclerosis and should be early diagnosed and treated.