Division of Academic Internal Medicine.
Division of Rheumatology.
Curr Opin Pulm Med. 2021 Sep 1;27(5):448-454. doi: 10.1097/MCP.0000000000000809.
Sarcoidosis is a poorly understood multisystem granulomatous disease that frequently involves the lungs but can affect any organ system. In this review, we summarize recent developments in the understanding of the immune dysregulation seen in sarcoidosis and propose a new expanded definition of human autoimmunity in sarcoidosis, and the implications it would have on treating sarcoidosis with targeted immunotherapy regimens in the future.
Sarcoidosis has been linked to infectious organisms like Mycobacterium and Cutibacterium, and certain manifestations of sarcoidosis have been linked to specific HLA alleles, but the overall pathogenesis remains uncertain. Sarcoidosis patients have similar patterns of cellular immune dysregulation seen in other autoimmune diseases like rheumatoid arthritis, and recent large-scale population studies show that sarcoidosis frequently presents with other autoimmune diseases.
Advancements in the understanding of sarcoidosis support its consideration as an autoimmune disease. Sarcoidosis patients carry a higher risk of comorbid autoimmune conditions which offers an excellent opportunity to further understand autoimmunity and explore biologic therapies in sarcoidosis treatment, and furthermore will better targeted immunotherapy regimens for sarcoidosis patients in the future.
结节病是一种病因不明的多系统肉芽肿性疾病,常累及肺部,但也可影响任何器官系统。在这篇综述中,我们总结了结节病中免疫失调的最新研究进展,并提出了一个新的扩展的结节病人类自身免疫的定义,以及它对未来用靶向免疫治疗方案治疗结节病的影响。
结节病与分枝杆菌和痤疮丙酸杆菌等感染性病原体有关,某些结节病表现与特定 HLA 等位基因有关,但总体发病机制仍不确定。结节病患者存在与类风湿关节炎等其他自身免疫性疾病相似的细胞免疫失调模式,最近的大规模人群研究表明,结节病常伴有其他自身免疫性疾病。
对结节病认识的进步支持将其视为一种自身免疫性疾病。结节病患者合并其他自身免疫性疾病的风险更高,这为进一步了解自身免疫和探索生物治疗在结节病治疗中的应用提供了极好的机会,并有望为未来的结节病患者提供更有针对性的免疫治疗方案。