Belperio John A, Shaikh Faisal, Abtin Fereidoun, Fishbein Michael C, Saggar Rajan, Tsui Edmund, Lynch Joseph P
The Division of Pulmonary and Critical Care Medicine, Holt and Jo Hickman Endowed Chair of Advanced Lung Disease and Lung Transplantation, Clinical Immunology, and Allergy, David Geffen School of Medicine at UCLA, 10833 Le Conte Ave, Room 37-131 CHS, Los Angeles, CA 90095, United States.
Department of Radiology, Thoracic and Interventional Section, David Geffen School of Medicine at UCLA, United States.
EClinicalMedicine. 2021 Jun 27;37:100966. doi: 10.1016/j.eclinm.2021.100966. eCollection 2021 Jul.
Sarcoidosis is a poorly understood granulomatous disease that involves the lungs and/or intrathoracic lymph nodes in more than 90% of cases. Although pulmonary sarcoidosis is the leading cause of mortality in this disease, this review focuses on three sites of extrapulmonary involvement (heart, nervous system, and eyes), since involvement of any of these sites can be catastrophic, leading to death, debilitation, or blindness. Patients with cardiac, ocular and neurosarcoidosis necessitate a multidisciplinary approach with careful and long-term follow-up. Prompt diagnosis with imaging and/or biopsy and treatment is required to avoid irreversible damage. Corticosteroids are the mainstay of therapy and are often associated with rapid and durable remissions. Immunosuppressive or biologic agents are reserved for patients failing or experiencing side effects from steroids. Managing sarcoidosis requires vigilance, judgement, and awareness of the vagaries of this fascinating disease.
结节病是一种了解甚少的肉芽肿性疾病，超过90%的病例累及肺部和/或胸内淋巴结。尽管肺部结节病是该疾病死亡的主要原因，但本综述聚焦于肺外受累的三个部位（心脏、神经系统和眼睛），因为这些部位中的任何一个受累都可能是灾难性的，会导致死亡、虚弱或失明。患有心脏、眼部和神经结节病的患者需要多学科方法以及仔细和长期的随访。需要通过影像学和/或活检进行快速诊断并治疗，以避免不可逆转的损害。皮质类固醇是治疗的主要药物，通常会带来快速且持久的缓解。免疫抑制药物或生物制剂则用于对类固醇治疗无效或出现副作用的患者。管理结节病需要警惕、判断力以及对这种迷人疾病变幻莫测之处的认识。
