Hassoun Paul M
Division of Pulmonary and Critical Care Medicine, Department of Medicine, Johns Hopkins University, Baltimore, Maryland 21205, USA.
Semin Respir Crit Care Med. 2009 Aug;30(4):429-39. doi: 10.1055/s-0029-1233312. Epub 2009 Jul 24.
Pulmonary arterial hypertension (PAH) may complicate diverse connective tissue diseases (CTDs) such as systemic sclerosis (SSc), systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), and mixed CTD (MCTD) and is an important cause of morbidity and mortality in this context. From a histological standpoint, the pulmonary vascular lesions in PAH complicating CTD are similar to those observed in idiopathic PAH (IPAH). However, prognosis and responsiveness to therapy are distinctly worse in PAH associated with CTD. PAH is most common in SSc (affecting up to 20% of patients with SSc), and most of the data regarding incidence, clinical features, and therapy of CTD-associated PAH are derived from patients with SSc (scleroderma). Although PAH may involve other CTDs, data are more limited (primarily small, uncontrolled series and case reports). Treatment strategies for PAH associated with CTD are similar to but are distinctly less effective than those for IPAH. Lung transplantation may be offered for a subset of patients with CTD-associated PAH, but results are less favorable than for IPAH due to comorbidities and specific complications associated with CTD (e.g., esophageal involvement, aspiration with SSc).
肺动脉高压（PAH）可能使多种结缔组织病（CTD）复杂化，如系统性硬化症（SSc）、系统性红斑狼疮（SLE）、类风湿关节炎（RA）和混合性结缔组织病（MCTD），并在这种情况下成为发病和死亡的重要原因。从组织学角度来看，PAH合并CTD时的肺血管病变与特发性PAH（IPAH）中观察到的病变相似。然而，与CTD相关的PAH的预后和对治疗的反应明显更差。PAH在SSc中最为常见（高达20%的SSc患者受影响），关于CTD相关PAH的发病率、临床特征和治疗的大多数数据都来自SSc（硬皮病）患者。虽然PAH可能累及其他CTD，但数据更为有限（主要是小型、无对照的系列研究和病例报告）。与CTD相关的PAH的治疗策略与IPAH相似，但效果明显较差。对于一部分CTD相关PAH患者可考虑进行肺移植，但由于与CTD相关的合并症和特定并发症（如食管受累、SSc患者的误吸），结果不如IPAH患者理想。
