8
views
0
recommends
+1 Recommend
0 collections
    0
    shares
      • Record: found
      • Abstract: found
      • Article: not found

      From granuloma to fibrosis: sarcoidosis associated pulmonary fibrosis

      research-article

      Read this article at

      ScienceOpenPublisherPMC
      Bookmark
          There is no author summary for this article yet. Authors can add summaries to their articles on ScienceOpen to make them more accessible to a non-specialist audience.

          Abstract

          Purpose of review

          Up to twenty percent of patients with sarcoidosis develop pulmonary fibrosis, transforming an often benign disease into a highly morbid and potentially fatal one. We highlight the fibrotic pulmonary sarcoidosis phenotype as an area of intense clinical and translational investigation, review recent developments in treatment, and provide a roadmap for future research in sarcoidosis associated pulmonary fibrosis.

          Recent findings

          Granulomatous inflammation in a lymphatic distribution is the hallmark finding of pulmonary sarcoidosis and the nidus for fibrosis. Recent research demonstrates that fibrotic sarcoidosis begins in the setting of persistent, uncontrolled inflammation, and is aided by pro-fibrotic genetic features and immune responses. Comparison to other fibrotic lung diseases also reveals key features that inform our understanding of common pathways in fibrosis.

          Summary

          Understanding the mechanisms of fibrotic transformation in sarcoidosis enhances clinical care and facilitates development of novel therapeutic options. The impact of these findings in fibrotic sarcoidosis may be amplified through application to other interstitial lung diseases marked by inflammatory to fibrotic transformation. Important aspects of clinical management of fibrotic sarcoidosis include surveillance for co-morbidities, such as pulmonary hypertension, airway disease, and infection, and assessment for pulmonary disease activity that may benefit from immunosuppression.

          Related collections

          Author and article information

          Journal
          9503765
          20595
          Curr Opin Pulm Med
          Curr Opin Pulm Med
          Current opinion in pulmonary medicine
          1070-5287
          1531-6971
          23 May 2017
          September 2016
          01 September 2017
          : 22
          : 5
          : 484-491
          Affiliations
          [1 ]University of Chicago, Section of Pulmonary and Critical Care Medicine
          [2 ]University of Pennsylvania, Division of Pulmonary, Allergy and Critical Care Medicine
          Author notes
          Corresponding Author: Catherine A. Bonham, University of Chicago, Pulmonary and Critical Care Medicine, 5841 South Maryland Avenue, Room W652, MC 6076, Chicago IL 60637, Phone: 773-834-3500, Fax: 773-702-6500, Catherine.bonham@ 123456uchospitals.edu
          Article
          PMC5532138 PMC5532138 5532138 nihpa877416
          10.1097/MCP.0000000000000301
          5532138
          27379967
          87024a82-e530-4b9d-b549-57ce00f819ee
          History
          Categories
          Article

          Bronchiectasis,Pulmonary fibrosis,Granulomatous inflammation,Sarcoidosis

          Comments

          Comment on this article