Université Paris-Sud, Centre National de Référence de l'Hypertension Artérielle Pulmonaire, Service de Pneumologie et Réanimation Respiratoire, Hôpital Antoine-Béclère, Assistance Publique, Hôpitaux de Paris, Clamart, France.
Clin Rev Allergy Immunol. 2011 Apr;40(2):104-16. doi: 10.1007/s12016-009-8194-2.
Pulmonary involvement is second in frequency only to esophageal involvement as a visceral complication of systemic sclerosis (SSc) and has surpassed renal involvement as the most common cause of death. Interstitial lung disease and pulmonary vascular disease, particularly pulmonary arterial hypertension, are the most commonly encountered types of lung involvement. Chronic aspiration, airway disease, neuromuscular weakness, extrinsic pulmonary restrictive pathology, pleural effusions, pneumothorax, and lung cancer cause clinically significant disease and occur commonly enough to be routinely considered in the assessment of the SSc patient with respiratory symptoms. Affected patients have a significantly worse prognosis than patients with SSc who are free of pulmonary involvement.
肺部受累是仅次于食管受累的系统性硬化症（SSc）内脏并发症，已超过肾脏受累成为最常见的死亡原因。间质性肺疾病和肺血管疾病，特别是肺动脉高压，是最常见的肺部受累类型。慢性吸入、气道疾病、神经肌肉无力、肺外限制性病理学、胸腔积液、气胸和肺癌导致临床上显著的疾病，并且发生的频率足以在评估有呼吸系统症状的 SSc 患者时常规考虑。受影响的患者预后明显比没有肺部受累的 SSc 患者差。
