Li Qiang, Sahhar Joanne, Littlejohn Geoffrey
Rheumatology Department, Monash Medical Centre, Melbourne, Victoria.
Aust Fam Physician. 2008 Oct;37(10):831-4.
Scleroderma (systemic sclerosis) is an uncommon connective tissue disease characterised by vascular, inflammatory and fibrotic dysfunction of multiple organ systems. Systemic sclerosis is often recognised late in the course of the disease.
This article outlines the clinical features of systemic sclerosis, in particular 'red flags' that indicate the presence of significant organ disease.
Common clinical features include Raynaud phenomenon and skin thickening, often with calcinosis and telangiectasia. These features should alert the physician to look for red flag features. In the general practice setting, early recognition of scleroderma will enable timely referral to specialist centres for regular screening and effective management of its many serious visceral complications.
硬皮病(系统性硬化症)是一种罕见的结缔组织疾病,其特征为多个器官系统出现血管、炎症和纤维化功能障碍。系统性硬化症在疾病进程中往往较晚才被识别。
本文概述了系统性硬化症的临床特征,特别是提示存在严重器官疾病的“警示信号”。
常见临床特征包括雷诺现象和皮肤增厚,常伴有钙质沉着和毛细血管扩张。这些特征应提醒医生留意警示信号特征。在基层医疗环境中,早期识别硬皮病将有助于及时转诊至专科中心,以便对其众多严重的内脏并发症进行定期筛查和有效管理。