Back to Search Start Over

The Pathogenesis of Pulmonary Sarcoidosis and Implications for Treatment.

Authors :
Patterson KC
Chen ES
Source :
Chest [Chest] 2018 Jun; Vol. 153 (6), pp. 1432-1442. Date of Electronic Publication: 2017 Dec 07.
Publication Year :
2018

Abstract

Thoracic sarcoidosis is the most common form of sarcoidosis, encompassing a heterogeneous group of patients with a wide range of clinical features and associated outcomes. The distinction between isolated thoracic lymphadenopathy and pulmonary involvement matters. Morbidity is often higher, and long-term outcomes are worse for the latter. Although inflammatory infiltrates in pulmonary sarcoidosis may resolve, persistent disease activity is common and can result in lung fibrosis. Given the distinct clinical features and natural history of pulmonary sarcoidosis, its pathogenesis may differ in important ways from other sarcoidosis manifestations. This review highlights recent advances in the pathogenesis of pulmonary sarcoidosis, including the nature of the sarcoidosis antigen, the role of serum amyloid A and other host factors that contribute to alterations in innate immunity, factors that shape adaptive T-cell profiles in the lung, and how these mechanisms influence the maintenance of granulomatous inflammation in sarcoidosis. We discuss questions raised by recent findings, including the role of innate immunity in the pathogenesis, the meaning of immune cell exhaustion, and mechanisms that may contribute to lung fibrosis in sarcoidosis. We conclude with a reflection on when and how immunosuppressive therapies may be helpful for pulmonary sarcoidosis, a consideration of nonpharmacologic management strategies, and a survey of potential novel therapeutic targets for this vexing disease.<br /> (Copyright © 2017 American College of Chest Physicians. All rights reserved.)

Details

Language :
English
ISSN :
1931-3543
Volume :
153
Issue :
6
Database :
MEDLINE
Journal :
Chest
Publication Type :
Academic Journal
Accession number :
29224832
Full Text :
https://doi.org/10.1016/j.chest.2017.11.030