Ungprasert Patompong, Ryu Jay H, Matteson Eric L
Clinical Epidemiology Unit, Department of Research and Development, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand.
Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Mayo Clinic, Rochester, MN.
Mayo Clin Proc Innov Qual Outcomes. 2019 Aug 2;3(3):358-375. doi: 10.1016/j.mayocpiqo.2019.04.006. eCollection 2019 Sep.
The focus of this review is current knowledge about the epidemiology, clinical manifestations, diagnosis, and treatment of both pulmonary sarcoidosis and extrapulmonary sarcoidosis. Although intrathoracic involvement is the hallmark of the disease, present in over 90% of patients, sarcoidosis can affect virtually any organ. Clinical presentations of sarcoidosis are diverse, ranging from asymptomatic, incidental findings to organ failure. Diagnosis requires the presence of noncaseating granuloma and compatible presentations after exclusion of other identifiable causes. Spontaneous remission is frequent, so treatment is not always indicated unless the disease is symptomatic or causes progressive organ damage/dysfunction. Glucocorticoids are the cornerstone of treatment of sarcoidosis even though evidence from randomized controlled studies is lacking. Glucocorticoid-sparing agents and biologic agents are often used as second- and third-line therapy for patients who do not respond to glucocorticoids or experience serious adverse effects.
本综述的重点是关于肺结节病和肺外结节病的流行病学、临床表现、诊断及治疗的现有知识。尽管胸腔受累是该疾病的标志,超过90%的患者存在此情况,但结节病实际上可累及任何器官。结节病的临床表现多种多样,从无症状的偶然发现到器官衰竭。诊断需要在排除其他可识别病因后存在非干酪样肉芽肿及相符的表现。疾病常可自发缓解,因此除非疾病有症状或导致进行性器官损害/功能障碍,否则不一定总是需要治疗。糖皮质激素是结节病治疗的基石,尽管缺乏随机对照研究的证据。对于对糖皮质激素无反应或出现严重不良反应的患者,糖皮质激素节省剂和生物制剂常作为二线和三线治疗药物。