Guillevin Loïc, Mouthon Luc
Médecine Interne, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, Université Paris Descartes, 27 rue du Faubourg Saint-Jacques, 75014 Paris, France.
Bull Acad Natl Med. 2011 Jan;195(1):79-90; discussion 90-2.
Pulmonary involvement in progressive systemic sclerosis can affect either the parenchyma or the vasculature. Both forms are fatal within a few months or years, due to respiratory and/or cardiac failure. Patients must be screened for pulmonary hypertension by ultrasonography, to be confirmed by right heart catheterization if necessary, as early diagnosis and immediate treatment improves the prognosis. The interstitial pulmonary disease progresses slowly, leading to respiratory failure, secondary pulmonary hypertension, and heart failure. Immunosuppressive treatments are disappointing but if prescribed early may prevent or delay the otherwise ineluctable aggravation. Pulmonary involvement threatens the vital and functional prognosis of patients with scleroderma, who may also develop several other complica- tions (especially infections and adverse effects of drugs) that are not dealt with here. This paper focuses on the most significant disease manifestations and current therapeutic options.
进行性系统性硬化症中的肺部受累可影响实质或血管系统。由于呼吸和/或心力衰竭，这两种形式在数月或数年内都是致命的。必须通过超声检查对患者进行肺动脉高压筛查，必要时通过右心导管检查进行确诊，因为早期诊断和及时治疗可改善预后。间质性肺病进展缓慢，会导致呼吸衰竭、继发性肺动脉高压和心力衰竭。免疫抑制治疗效果不佳，但早期使用可能预防或延缓原本不可避免的病情加重。肺部受累威胁着硬皮病患者的生命和功能预后，患者还可能出现其他几种并发症（尤其是感染和药物不良反应），本文对此不作讨论。本文重点关注最重要的疾病表现和当前的治疗选择。
