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一例弥漫性皮肤系统性硬化症患者发生的早发型血压正常的硬皮病肾危象。

A case of early onset normotensive scleroderma renal crisis in a patient with diffuse cutaneous systemic sclerosis.

作者信息

Bashandy Hany G, Javillo Jason S, Gambert Steven R

机构信息

Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, MD 21215, USA.

出版信息

South Med J. 2006 Aug;99(8):870-2. doi: 10.1097/01.smj.0000229871.68911.67.

Abstract

Scleroderma renal crisis (SRC) has classically been defined as a new onset of accelerated arterial hypertension associated with a rapid increase in serum creatinine concentration and/or microangiopathic hemolytic anemia. SRC occurs in approximately 20% of patients with systemic sclerosis who have diffuse cutaneous manifestations. In addition, 10% of reported cases of SRC with diffuse cutaneous involvement have normal blood pressures; in the majority of these cases, SRC occurs after treatment with corticosteroids. We describe a patient who presented with an early onset SRC in the setting of diffuse cutaneous systemic sclerosis in evolution without prior accelerated arterial hypertension or corticosteroid use.

摘要

硬皮病肾危象(SRC)传统上被定义为新发的加速性动脉高血压,伴有血清肌酐浓度迅速升高和/或微血管病性溶血性贫血。SRC发生于约20%有弥漫性皮肤表现的系统性硬化症患者中。此外,10%报告的弥漫性皮肤受累的SRC病例血压正常;在这些病例中的大多数,SRC发生于使用皮质类固醇治疗后。我们描述了一名在弥漫性皮肤系统性硬化症进展过程中出现早期SRC的患者,该患者既往无加速性动脉高血压或未使用过皮质类固醇。

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