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

被引：5

作者：

Cocconcelli, Elisabetta ^{[1
]}

Bernardinello, Nicol ^{[1
]}

Castelli, Gioele ^{[1
]}

Petrarulo, Simone ^{[1
]}

Bellani, Serena ^{[1
]}

Saetta, Marina ^{[1
]}

Spagnolo, Paolo ^{[1
]}

Balestro, Elisabetta ^{[1
]}

机构：

[1] Padova Univ Hosp, Resp Dis Unit, Dept Cardiac Thorac Vasc Sci & Publ Hlth, I-35128 Padua, Italy

来源：

INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES | 2023年 / 24卷 / 13期

关键词：

sarcoidosis; advanced pulmonary fibrosis; signaling; molecular pathway; T-CELLS; TGF-BETA; LUNG-DISEASE; MACROPHAGES; CYTOKINES; UPDATE; POLYMORPHISMS; EPIDEMIOLOGY; HYPERTENSION; PATHOGENESIS;

D O I：

10.3390/ijms241310767

中图分类号：

Q5 [生物化学]; Q7 [分子生物学];

学科分类号：

071010 ; 081704 ;

摘要：

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.

引用

页数：14

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