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      Spectrum of Fibrotic Lung Diseases

      1 , 1
      New England Journal of Medicine
      Massachusetts Medical Society

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          Nintedanib in Progressive Fibrosing Interstitial Lung Diseases

          Preclinical data have suggested that nintedanib, an intracellular inhibitor of tyrosine kinases, inhibits processes involved in the progression of lung fibrosis. Although the efficacy of nintedanib has been shown in idiopathic pulmonary fibrosis, its efficacy across a broad range of fibrosing lung diseases is unknown.
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            Fibrosis--a common pathway to organ injury and failure.

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              Sarcoidosis.

              Sarcoidosis is a systemic disease of unknown cause that is characterised by the formation of immune granulomas in various organs, mainly the lungs and the lymphatic system. Studies show that sarcoidosis might be the result of an exaggerated granulomatous reaction after exposure to unidentified antigens in individuals who are genetically susceptible. Several new insights have been made, particularly with regards to the diagnosis and care of some important manifestations of sarcoidosis. The indications for endobronchial ultrasound in diagnosis and for PET in the assessment of inflammatory activity are now better specified. Recognition of unexplained persistent disabling symptoms, fatigue, small-fibre neurological impairment, cognitive failure, and changes to health state and quality of life, has improved. Mortality in patients with sarcoidosis is higher than that of the general population, mainly due to pulmonary fibrosis. Predicted advances for the future are finding the cause of sarcoidosis, and the elucidation of relevant biomarkers, reliable endpoints, and new efficient treatments, particularly in patients with refractory sarcoidosis, lung fibrosis, and those with persistent disabling symptoms. Copyright © 2014 Elsevier Ltd. All rights reserved.
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                Author and article information

                Contributors
                (View ORCID Profile)
                (View ORCID Profile)
                Journal
                New England Journal of Medicine
                N Engl J Med
                Massachusetts Medical Society
                0028-4793
                1533-4406
                September 03 2020
                September 03 2020
                : 383
                : 10
                : 958-968
                Affiliations
                [1 ]From the Center for Interstitial Lung Diseases and Sarcoidosis, Department of Respiratory Medicine, Erasmus MC–University Medical Center Rotterdam, Rotterdam, the Netherlands (M.W.); and the Department of Respiratory Medicine, National Coordinating Reference Center for Rare Pulmonary Diseases, Louis Pradel Hospital, and Claude Bernard University — both in Lyon, France (V.C.).
                Article
                10.1056/NEJMra2005230
                32877584
                ef1de716-a1b2-45a5-b5c5-feb4581362f1
                © 2020
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