Department of Rheumatic and Immunologic Diseases, Orthopedic and Rheumatology Institute, Cleveland Clinic, Cleveland, OH 44195, USA.
Semin Arthritis Rheum. 2011 Aug;41(1):19-37. doi: 10.1016/j.semarthrit.2010.08.004. Epub 2010 Nov 2.
To discuss the clinical subtypes, pathogenesis, pathology, diagnostic evaluation, treatment options, and prognosis of pulmonary hypertension in systemic sclerosis (SSc-PH) and highlight its fundamental differences from idiopathic pulmonary arterial hypertension (IPAH).
A Medline search for articles published between January 1969 and June 2010 was conducted using the following keywords: scleroderma, systemic sclerosis, pulmonary hypertension, pulmonary arterial hypertension, pulmonary veno-occlusive disease, pathogenesis, pathology, investigation, treatment, and prognosis. The essential differences from IPAH in pathogenesis and histopathologic findings were highlighted and the limitations of some of the investigations used were emphasized. The differences in response to currently accepted therapy and prognosis were also reviewed.
In scleroderma, pulmonary hypertension can be present in isolation or along with interstitial lung disease and left heart disease. In SSc-PH, the unique histopathologic findings in the lungs include intimal fibrosis, absence of plexiform lesions, and a high prevalence of pulmonary veno-occlusive disease-like lesions. Both "6-minute walk test" and NT-proBNP have their limitations in the evaluation of SSc-PH. For treatment, calcium channel blockers are ineffective and anticoagulation should be used with caution. Currently approved therapies are not as effective and prognosis is much worse in SSc-PH compared with IPAH.
SSc-PH is a complex condition with poorer response to therapy and worse outcome compared with that of IPAH. Recent findings have shed some light about the pathophysiology and pathogenesis of SSc-PH. Further research in this area is warranted to better understand the complex pathogenesis and devise better therapeutic strategies.
讨论系统性硬皮病（SSc）相关肺动脉高压（SSc-PH）的临床亚型、发病机制、病理学、诊断评估、治疗选择和预后，并强调其与特发性肺动脉高压（IPAH）的根本区别。
通过使用以下关键词，对 1969 年 1 月至 2010 年 6 月间发表的文章进行了 Medline 检索：硬皮病、系统性硬化症、肺动脉高压、肺动脉高血压、肺静脉闭塞病、发病机制、病理学、调查、治疗和预后。强调了在发病机制和组织病理学发现方面与 IPAH 的重要区别，并强调了一些用于诊断的检查方法的局限性。还回顾了对目前公认的治疗方法的反应和预后的差异。
在硬皮病中，肺动脉高压可单独存在，也可与间质性肺病和左心疾病同时存在。在 SSc-PH 中，肺部的独特组织病理学表现包括内膜纤维化、无丛状病变，以及高发性肺静脉闭塞病样病变。“6 分钟步行试验”和 NT-proBNP 在评估 SSc-PH 时都有其局限性。对于治疗，钙通道阻滞剂无效，抗凝治疗应谨慎使用。目前批准的治疗方法在 SSc-PH 中不如 IPAH 有效，预后也差得多。
与 IPAH 相比，SSc-PH 是一种更为复杂的疾病，其治疗反应和预后较差。最近的研究结果揭示了 SSc-PH 的病理生理学和发病机制的一些方面。需要在这一领域进行进一步的研究，以更好地了解复杂的发病机制并制定更好的治疗策略。
