Kirsten D
Ambulanz für Seltene Lungenkrankheiten und Sarkoidose an der LungenClinic Grosshansdorf, Großhansdorf, Germany.
Dtsch Med Wochenschr. 2013 Mar;138(11):537-41. doi: 10.1055/s-0032-1332898. Epub 2013 Mar 5.
Sarcoidosis is a multisystemic granulomatous disorder of unknown origin, which can involve multiple organs. However the lymph node and lung manifestations dominate. Most frequent symptoms are cough, fatigue, dyspnea and exercise limitations. About one third of the patients have no symptoms. The lung function test is mostly restrictive. The clinical course is very variable, spontaneous remissions occur in about 60 %. Acute sarcoidosis is a highly inflammatory disease with a very good prognosis even without steroid therapy. Chronic pulmonary sarcoidosis can lead to progressive loss of lung function. Indications to therapy are severe symptoms and loss of lung function as well as progressive lung involvement with fibrosis pattern. In chronic disease corticosteroids are effective in the majority of patients. Other immunosuppressives, cytotoxic and immunomodulatory agents are reserved for patients with severe side-effects to steroid-therapy or for patients, who cannot maintained on reasonable low doses of steroids. Lung transplantation is an option for life-threatening cases failing medical therapy.
结节病是一种病因不明的多系统肉芽肿性疾病，可累及多个器官。然而，淋巴结和肺部表现最为常见。最常见的症状是咳嗽、疲劳、呼吸困难和运动受限。约三分之一的患者没有症状。肺功能测试大多显示为限制性通气功能障碍。临床病程差异很大，约60%的患者会自发缓解。急性结节病是一种高度炎症性疾病，即使不进行类固醇治疗，预后也很好。慢性肺部结节病可导致肺功能逐渐丧失。治疗指征为严重症状、肺功能丧失以及肺部出现进行性纤维化改变。在慢性病中，大多数患者使用皮质类固醇有效。其他免疫抑制剂、细胞毒性药物和免疫调节剂则用于对类固醇治疗有严重副作用的患者或无法维持合理低剂量类固醇治疗的患者。对于药物治疗无效的危及生命的病例，肺移植是一种选择。
