Lynch Joseph P, Ma Yan Ling, Koss Michael N, White Eric S
Division of Pulmonary, Critical Care Medicine, and Hospitalists, Department of Internal Medicine, The David Geffen School of Medicine at UCLA, Los Angeles, California 90095, USA.
Semin Respir Crit Care Med. 2007 Feb;28(1):53-74. doi: 10.1055/s-2007-970333.
Sarcoidosis, a granulomatous disorder of unknown etiology, characteristically involves multiple organs. However, pulmonary manifestations typically dominate. Chest radiographs are abnormal in 85 to 95% of patients. Abnormalities in pulmonary function tests are common and may be associated with cough, dyspnea, and exercise limitation. However, one third or more of patients are asymptomatic, with incidental abnormalities on chest radiographs. The clinical course and expression of pulmonary sarcoidosis are variable. Spontaneous remissions occur in nearly two thirds of patients. The course is chronic in up to 30% of patients. Chronic pulmonary sarcoidosis may result in progressive (sometimes life-threatening) loss of lung function. Fatalities ascribed to sarcoidosis occur in 1 to 4% of patients. Although the impact of treatment is controversial, corticosteroids may be highly effective in some patients. Immunosuppressive, cytotoxic, or immunomodulatory agents are reserved for patients failing or experiencing adverse effects from corticosteroids. Lung transplantation is a viable option for patients with life-threatening disease failing medical therapy.
结节病是一种病因不明的肉芽肿性疾病，其特征是累及多个器官。然而，肺部表现通常最为突出。85%至95%的患者胸部X线片异常。肺功能测试异常很常见，可能与咳嗽、呼吸困难和运动受限有关。然而，三分之一或更多的患者没有症状，胸部X线片上有偶然发现的异常。肺部结节病的临床病程和表现各不相同。近三分之二的患者会自发缓解。高达30%的患者病程呈慢性。慢性肺部结节病可能导致肺功能进行性（有时危及生命）丧失。结节病导致的死亡发生在1%至4%的患者中。尽管治疗的效果存在争议，但皮质类固醇在一些患者中可能非常有效。免疫抑制、细胞毒性或免疫调节药物仅用于对皮质类固醇治疗无效或出现不良反应的患者。对于药物治疗无效的危及生命的疾病患者，肺移植是一种可行的选择。
