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Antifibrotic drugs for pulmonary sarcoidosis: A treatment in search of an indication

Published:March 22, 2021DOI:https://doi.org/10.1016/j.rmed.2021.106371
      The most common causes of disability and death from sarcoidosis are directly or indirectly related to complications of fibrotic lung disease [
      • Judson M.A.
      Strategies for identifying pulmonary sarcoidosis patients at risk for severe or chronic disease.
      ]. Fibrosis develops in up to 20% of pulmonary sarcoidosis patients [
      • Moller D.R.
      Pulmonary fibrosis of sarcoidosis. New approaches, old ideas.
      ,
      • Judson M.A.
      • Boan A.D.
      • Lackland D.T.
      The clinical course of sarcoidosis: presentation, diagnosis, and treatment in a large white and black cohort in the United States.
      ] and may result in end-stage parenchymal lung disease [
      • Baughman R.P.
      • Lower E.E.
      Frequency of acute worsening events in fibrotic pulmonary sarcoidosis patients.
      ]. Fibrotic pulmonary sarcoidosis is associated with distortion of airways leading to airflow obstruction, bronchiectasis, and increased episodes of clinical worsening that are often related to pulmonary infections [
      • Baughman R.P.
      • Lower E.E.
      Frequency of acute worsening events in fibrotic pulmonary sarcoidosis patients.
      ,
      • Hennebicque A.S.
      • Nunes H.
      • Brillet P.Y.
      • Moulahi H.
      • Valeyre D.
      • Brauner M.W.
      CT findings in severe thoracic sarcoidosis.
      ,
      • Lewis M.M.
      • Mortelliti M.P.
      • Yeager Jr., H.
      • Tsou E.
      Clinical bronchiectasis complicating pulmonary sarcoidosis: case series of seven patients.
      ,
      • Polychronopoulos V.S.
      • Prakash U.B.
      Airway involvement in sarcoidosis.
      ]. Sarcoidosis associated pulmonary hypertension is caused by a variety of mechanisms, but the most common of these is distortion of the pulmonary vasculature from fibrosis [
      • Handa T.
      • Nagai S.
      • Miki S.
      • et al.
      Incidence of pulmonary hypertension and its clinical relevance in patients with sarcoidosis.
      ,
      • Baughman R.P.
      • Shlobin O.A.
      • Wells A.U.
      • et al.
      Clinical features of sarcoidosis associated pulmonary hypertension: results of a multi-national registry.
      ]. Mycetoma that are associated with pulmonary sarcoidosis occur almost exclusively in those with fibrocystic disease [
      • Uzunhan Y.
      • Nunes H.
      • Jeny F.
      • et al.
      Chronic pulmonary aspergillosis complicating sarcoidosis.
      ] where they may cause life-threatening hemoptysis [
      • Kravitz J.N.
      • Berry M.W.
      • Schabel S.I.
      • Judson M.A.
      A modern series of percutaneous intracavitary instillation of amphotericin B for the treatment of severe hemoptysis from pulmonary aspergilloma.
      ]. All the aforementioned manifestations of fibrotic pulmonary sarcoidosis commonly result in severe quality of life impairment and are potentially life-threatening [
      • Uzunhan Y.
      • Nunes H.
      • Jeny F.
      • et al.
      Chronic pulmonary aspergillosis complicating sarcoidosis.
      ,
      • Kravitz J.N.
      • Berry M.W.
      • Schabel S.I.
      • Judson M.A.
      A modern series of percutaneous intracavitary instillation of amphotericin B for the treatment of severe hemoptysis from pulmonary aspergilloma.
      ,
      • Kirkil G.
      • Lower E.E.
      • Baughman R.P.
      Predictors of mortality in pulmonary sarcoidosis.
      ,
      • Walsh S.L.
      • Wells A.U.
      • Sverzellati N.
      • et al.
      An integrated clinicoradiological staging system for pulmonary sarcoidosis: a case-cohort study.
      ].

      Keywords

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