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无硬皮病的系统性硬化症:硬皮病肾危象中的一种不寻常表现。

Systemic sclerosis sine scleroderma: an unusual presentation in scleroderma renal crisis.

作者信息

Molina J F, Anaya J M, Cabrera G E, Hoffman E, Espinoza L R

机构信息

Department of Medicine, Louisiana State University Medical Center, New Orleans 70112-2822, USA.

出版信息

J Rheumatol. 1995 Mar;22(3):557-60.

PMID:7783082
Abstract

Renal involvement in systemic sclerosis (SSc) has a negative influence on prognosis. Cases of SSc sine scleroderma have been reported in which organ failure occurred but was not accompanied by cutaneous damage, which in some instances did develop later. We describe a patient who, after 6 months of symmetric polyarthritis, developed rapid progressive renal failure without skin changes. A diagnosis of scleroderma renal crisis, confirmed histologically, was made. Anti-RNAP III antibodies were positive. The patient developed typical scleroderma skin changes after renal failure. Despite treatment, SSc advanced to fatal endstage renal disease.

摘要

系统性硬化症(SSc)中的肾脏受累对预后有负面影响。曾有硬皮病无硬皮症病例的报道,这些病例出现了器官衰竭,但未伴有皮肤损害,其中一些病例后来确实出现了皮肤损害。我们描述了一名患者,在出现6个月的对称性多关节炎后,出现了快速进展的肾衰竭,且无皮肤改变。经组织学确诊为硬皮病肾危象。抗RNA聚合酶III抗体呈阳性。该患者在肾衰竭后出现了典型的硬皮病皮肤改变。尽管进行了治疗,SSc仍进展至致命的终末期肾病。

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