Judson M A
J S C Med Assoc. 2000 Jan;96(1):9-17.
Pulmonary sarcoidosis has a variable natural course from an asymptomatic state to a progressive life-threatening condition. Radiographic abnormalities are frequently an important clue to the diagnosis. The diagnosis usually requires a tissue biopsy that demonstrates noncaseating granulomas that cannot be ascribed to another clinical condition. The lung may be biopsied, but extrapulmonary sites may be biopsied for the diagnosis if such sites are involved with disease. When the lung is biopsied, a transbronchial lung biopsy with a flexible bronchoscope is the procedure of choice, even if the chest radiograph shows thoracic adenopathy alone without obvious parenchymal infiltration. On occasion the diagnosis can be made on clinical grounds without biopsy when the presentation is highly specific for sarcoidosis, such as Lofgren's Syndrome. Treatment for pulmonary sarcoidosis has not been standardized. Since many patients have spontaneous remissions and the benefits of therapy do not affect the long-term outcome, therapy is reserved for patients with severe or progressive pulmonary symptoms and/or pulmonary dysfunction. Corticosteroids are the primary therapy for pulmonary sarcoidosis. Corticosteroid therapy involves six phases: initial high dose, taper to a maintenance dose, a maintenance dose, taper off corticosteroids, monitor off therapy, and treatment of relapse if it occurs.
肺结节病的自然病程变化多样，可从无症状状态发展为危及生命的进行性疾病。影像学异常常常是诊断的重要线索。诊断通常需要组织活检，以显示非干酪样肉芽肿，且该肉芽肿不能归因于其他临床情况。可对肺进行活检，但如果肺外部位有病变累及，也可对这些部位进行活检以明确诊断。当对肺进行活检时，即使胸部X线片仅显示胸内淋巴结肿大而无明显实质浸润，首选的方法也是使用可弯曲支气管镜进行经支气管肺活检。有时，当临床表现对结节病具有高度特异性时，如洛弗格伦综合征，无需活检即可根据临床情况作出诊断。肺结节病的治疗尚未标准化。由于许多患者会自发缓解，且治疗的益处并不影响长期预后，因此治疗仅适用于有严重或进行性肺部症状和/或肺功能障碍的患者。皮质类固醇是治疗肺结节病的主要药物。皮质类固醇治疗包括六个阶段：初始高剂量、逐渐减量至维持剂量、维持剂量、停用皮质类固醇、停药后监测，以及复发时的治疗。
