Division of Pulmonary and Critical Care Medicine, Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland 21224, USA.
Curr Opin Rheumatol. 2010 Nov;22(6):696-703. doi: 10.1097/BOR.0b013e32833dfbd8.
Systemic sclerosis (SSc), a collagen vascular disease characterized by diverse organ system involvement, is associated with serious cardiac complications. Cardiac symptoms are much less frequent than autopsy-demonstrated cardiac involvement. Although frequent calls for early diagnosis have been made, validated strategies for assessment of scleroderma cardiac disease are not yet well established, mainly because the right ventricle, a common target of the disease, presents considerable obstacles to simple and reliable appraisal of its structure and function. This article reviews the current information about cardiac dysfunction in scleroderma, with special emphasis on its detection and prognostic implications.
Cardiac involvement is a harbinger of poor prognosis in patients with SSc. Recent progress in its diagnosis has been made with the availability of more sophisticated diagnostic tools.
Early detection of scleroderma heart disease will allow exploration of novel therapies with potential positive impact on the quality of life and life expectancy of this patient population.
系统性硬化症（SSc）是一种以多种器官系统受累为特征的胶原血管疾病，与严重的心脏并发症相关。心脏症状比尸检证实的心脏受累少见得多。尽管经常呼吁早期诊断，但尚未建立评估硬皮病心脏病的经过验证的策略，主要是因为疾病的常见靶器官右心室对其结构和功能的简单可靠评估存在相当大的障碍。本文综述了硬皮病心脏功能障碍的最新信息，特别强调了其检测和预后意义。
心脏受累是 SSc 患者预后不良的先兆。随着更复杂的诊断工具的出现，其诊断方面取得了新的进展。
早期发现硬皮病心脏病将允许探索新的治疗方法，有可能对这一患者群体的生活质量和预期寿命产生积极影响。
