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系统性硬化症(硬皮病)中的肾病，包括硬皮病肾危象

Authors
John Varga, MD
Andrew Z Fenves, MD
Section Editors
Richard J Glassock, MD, MACP
Gary C Curhan, MD, ScD
Deputy Editor
Alice M Sheridan, MD
Translators
张明, 副主任医师

引言

系统性硬化症(systemic sclerosis, SSc，硬皮病)的病理特征是：不受控制的胶原沉积和以血管壁增厚和血管腔狭窄为特征的广泛血管病变。 (参见“Pathogenesis of systemic sclerosis (scleroderma)”)

硬皮病可能仅累及皮肤和皮下组织，也可能累及全身。当特征性皮肤病变伴有内脏器官受累时使用SSc这一术语。 (参见“成人系统性硬化症(硬皮病)的临床表现概述”)

肾受累在SSc患者中很常见，最严重的表现是硬皮病肾危象(scleroderma renal crisis, SRC)，其发生于少数患者中。 (参见下文‘肾病的患病率和表现’)

SRC有以下3个主要特征(参见下文‘临床特征’)：

●突然发生的中度至重度高血压，通常伴血浆肾素活性增高

                               

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Literature review current through: 2017-06 . | This topic last updated: 2016-06-16.
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