Molecular Mechanism in the Development of Pulmonary Fibrosis in Patients with Sarcoidosis

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.

Automated summary

Sarcoidosis is a disease where granulomas form in various organs, particularly the lungs. The course of the disease can be unpredictable, with some patients experiencing spontaneous remission while others develop chronic lung disease. The mechanism leading to fibrosis in advanced pulmonary sarcoidosis is still unknown, but recent studies have shed light on the genetic and immune factors involved. This review aims to summarize the clinical-prognostic characteristics and molecular pathways associated with the development of advanced pulmonary sarcoidosis.