Nunes H, Maurer C, Naccache J M, Girard F, Brauner M, Valeyre D
Fédération des Maladies Respiratoires et Thoraciques de l'Adulte, Hôpital Avicenne, 125, rue de Stalingrad, 93009 Bobigny.
Ann Med Interne (Paris). 2001 Mar;152(2):96-102.
Mediastinal and pulmonary localizations are found in 90% of al patients with sarcoidosis. In half the cases, the disease is not severe and is reversible without treatment. In the other half of cases, early or late respiratory complications can be seen. Early complications include subacute respiratory insufficiency by interstitial lung disease or by bronchial airway obstruction. Among late complications, the most frequent is pulmonary fibrosis. Four computed tomography patterns are found with variable functional impairments and course. Chronic obstructive respiratory insufficiency can be the consequence of specific bronchial lesions or pulmonary fibrosis surrounding proximal bronchi. Cor pulmonale is seen in 5% of cases. Aspergilloma seen in fibroemphysematous lesions can be the cause of major hempoptysis. Respiratory complications account for half of the 5% of deaths due to sarcoidosis. Respiratory complications are most often seen in radiographic stage III and IV disease. Treatments, mainly corticosteroids, only exert a suspensive effect but reduce the incidence and severity of respiratory manifestations. The gain obtained by treatment depends on the choice of the best time of institution and on the quality of monitoring. Lung transplantation is useful in most severe cases unresponsive to medical treatment.
90%的结节病患者存在纵隔和肺部病变。半数病例中，疾病并不严重，未经治疗也可逆转。在另一半病例中，可出现早期或晚期呼吸并发症。早期并发症包括间质性肺病或支气管气道阻塞导致的亚急性呼吸功能不全。晚期并发症中，最常见的是肺纤维化。根据功能损害和病程的不同，可发现四种计算机断层扫描模式。慢性阻塞性呼吸功能不全可能是特定支气管病变或近端支气管周围肺纤维化的结果。5%的病例会出现肺心病。在纤维性肺气肿病变中发现的曲菌球可能是严重咯血的原因。呼吸并发症占结节病所致死亡的5%的一半。呼吸并发症最常出现在影像学III期和IV期疾病中。治疗主要是使用皮质类固醇，仅起缓解作用，但可降低呼吸表现的发生率和严重程度。治疗所获得的益处取决于最佳治疗时机的选择和监测质量。在大多数对药物治疗无反应的严重病例中，肺移植是有效的。
