Clinical approach to scleroderma.
Clinical approach to scleroderma.
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硬皮病的临床方法。
DOI：
10.1016/s1085-5629(98)80016-3
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发表时间：
1998
期刊：
影响因子：
--
通讯作者：
White,B
中科院分区：
文献类型：
--
作者：
White,B
系统性硬化症（SSc）是一种发病率和死亡率差异很大的异质性疾病。尽管如此，可以根据疾病最初几年内皮肤和内脏器官受累的严重程度来估计个体患者的未来临床病程。有限皮肤SSc（ISSc）患者肘部或膝盖以下皮肤增厚，可能累及面部和颈部。患有这种亚型的SSc的患者具有雷诺现象、指溃疡和食管功能障碍。发生间质性肺病或肺动脉高压的ISSc患者发病率和死亡率显著升高。弥漫性皮肤SSc（dSSc）患者的肘部和膝盖以上或躯干皮肤增厚。这些患者发病较突然，常伴有全身症状和关节痛。严重的心脏、肺、肠道和肾脏受累，如果发生，往往在疾病的前5年内发展，特别是在前几年内。有明显内脏器官受累的患者比无内脏器官受累的患者预后差。最初的病史、体格检查和实验室检查的目的是将硬皮病的类型分类为ISSc或dSSc，估计疾病持续时间，并确定器官受累的程度和严重程度。SSc的治疗是基于器官的。治疗可降低与雷诺现象、指溃疡、食管动力障碍、食管反流、肠动力障碍、关节痛、肌炎和肺动脉高压相关的发病率。治疗可以稳定间质性肺病伴肺泡炎患者的肺功能，稳定肾危象患者的肾功能。SSc患者的总体预后似乎正在改善。应考虑将早期dSSc患者纳入潜在疾病缓解疗法的方案检测。
Systemic sclerosis (SSc) is a heterogenous disease with a morbidity and mortality that varies widely. Nonetheless, the future clinical course of an individual patient can be estimated based on the severity of skin and internal organ involvement within the first several years of the disease. Patients with limited cutaneous SSc (ISSc) have skin thickening below the elbows or knees and may have face and neck involvement. Patients with this subtype of SSc have Raynaud's phenomenon, digital ulcers, and esophageal dysfunction. Significant morbidity and mortality arises in those patients with ISSc who develop interstitial lung disease or pulmonary artery hypertension. Patients with diffuse cutaneous SSc (dSSc) have skin thickening above the elbows and knees or on the trunk. These patients have a more abrupt onset of disease, often with constitutional symptoms and arthalgias. Severe heart, lung, gut, and renal involvement, if it occurs, tends to develop within the first 5 years of disease, especially within the first several years. Patients with significant internal organ involvement have a poorer prognosis than patients who do not. The goals of the initial history and physical and laboratory examinations are to classify the type of scleroderma as ISSc or dSSc, to estimate disease duration, and to define the extent and severity of organ involvement. Treatment of SSc is organ based. Treatment may reduce morbidity associated with Raynaud's phenomenon, digital ulcers, esophageal dysmotility, esophageal reflux, gut dysmotility, arthralgias, myositis, and pulmonary artery hypertension. Therapy may stabilize lung function in patients with interstitial lung disease with alveolitis and stabilize renal function in patients with renal crisis. The overall prognosis for patients with SSc appears to be improving. Patients with early dSSc should be considered for enrollment onto protocol testing of potential disease-modifying therapies.