Fibrotic Pulmonary Sarcoidosis

Clin Chest Med. 2024 Mar;45(1):199-212. doi: 10.1016/j.ccm.2023.08.011. Epub 2023 Sep 4.

Abstract

Fibrotic pulmonary sarcoidosis (fPS) affects about 20% of patients. fPS carries a significant morbidity and mortality. However, its prognosis is highly variable, depending mainly on fibrosis extent, functional impairment severity, and the development of pulmonary hypertension. Moreover, fPS outcomes are also influenced by several other complications, including acute exacerbations, and infections. fPS natural history is unknown, in particular regarding the risk of progressive self-sustaining fibrosis. The management of fPS is challenging, including anti-inflammatory treatment if granulomatous activity persists, rehabilitation, and in highly selected patients antifibrotic treatment and lung transplantation.

Keywords: Fibrosis; Granuloma; Morbidity; Mortality; Progression.

Publication types

  • Review

MeSH terms

  • Humans
  • Hypertension, Pulmonary* / complications
  • Hypertension, Pulmonary* / therapy
  • Lung Transplantation* / adverse effects
  • Prognosis
  • Pulmonary Fibrosis* / complications
  • Pulmonary Fibrosis* / therapy
  • Sarcoidosis* / complications
  • Sarcoidosis, Pulmonary* / complications
  • Sarcoidosis, Pulmonary* / therapy