Cordier Jean-François
Service de Pneumologie, Centre de référence des maladies rares pulmonaires, Hôpital Cardiovasculaire et Pneumologique Louis Pradel 69677 Lyon (Bron) Cedex.
Bull Acad Natl Med. 2009 Nov;193(8):1911-8; discussion 1918-9.
Among connective tissue diseases, pulmonary arterial hypertension (PAH) is frequently associated with systemic sclerosis and systemic lupus erythematosus. PAH is less common in mixed connective tissue diseases and Sjögren's syndrome, and rare in rheumatoid arthritis. PAH in systemic sclerosis may be either isolated (prevalence about 8%) or associated with interstitial lung disease. Echocardiographic screening for PAH is worthwhile in patients with systemic sclerosis, especially as treatments for idiopathic PAH (endothelin receptor antagonists, phosphodiesterase-5 inhibitors, and prostanoids) are effective in this setting. The prevalence of PAH among patients with systemic lupus erythematosus is poorly known; immunosuppressive treatment is sometimes effective by itself but most patients benefit from PAH treatment. PAH associated with connective tissue diseases has a worse prognosis than idiopathic PAH.
在结缔组织病中，肺动脉高压（PAH）常与系统性硬化症和系统性红斑狼疮相关。PAH在混合性结缔组织病和干燥综合征中较少见，在类风湿关节炎中罕见。系统性硬化症中的PAH可能是孤立性的（患病率约8%）或与间质性肺疾病相关。对系统性硬化症患者进行超声心动图筛查PAH是值得的，特别是因为特发性PAH的治疗（内皮素受体拮抗剂、磷酸二酯酶-5抑制剂和前列腺素）在这种情况下是有效的。系统性红斑狼疮患者中PAH的患病率尚不清楚；免疫抑制治疗有时本身有效，但大多数患者受益于PAH治疗。与结缔组织病相关的PAH预后比特发性PAH更差。
