Respiratory Disease Unit, Department of Cardiac, Thoracic, Vascular Sciences and Public Health, Padova University Hospital, 35128 Padova, Italy.
Int J Mol Sci. 2023 Jun 28;24(13):10767. doi: 10.3390/ijms241310767.
Sarcoidosis is a multisystemic disease of unknown etiology characterized by the formation of granulomas in various organs, especially lung and mediastinal hilar lymph nodes. The clinical course and manifestations are unpredictable: spontaneous remission can occur in approximately two thirds of patients; up to 20% of patients have chronic course of the lung disease (called advanced pulmonary sarcoidosis, APS) resulting in progressive loss of lung function, sometimes life-threatening that can lead to respiratory failure and death. The immunopathology mechanism leading from granuloma formation to the fibrosis in APS still remains elusive. Recent studies have provided new insights into the genetic factors and immune components involved in the clinical manifestation of the disease. In this review we aim to summarize the clinical-prognostic characteristics and molecular pathways which are believed to be associated with the development of APS.
结节病是一种病因不明的多系统疾病，其特征是在各种器官中形成肉芽肿，特别是肺和纵隔门淋巴结。其临床病程和表现不可预测：大约三分之二的患者会自发缓解；多达 20%的患者有肺部疾病的慢性病程（称为进展性肺结节病，APS），导致肺功能进行性丧失，有时危及生命，可导致呼吸衰竭和死亡。从肉芽肿形成到 APS 纤维化的免疫病理机制仍然难以捉摸。最近的研究为涉及疾病临床表现的遗传因素和免疫成分提供了新的见解。在这篇综述中，我们旨在总结与 APS 发展相关的临床预后特征和分子途径。
