• Vincent Cottin, Nikhil A Hirani, David L Hotchkin, Anoop M Nambiar, Takashi Ogura, María Otaola, Dirk Skowasch, Jong Sun Park, Hataya K Poonyagariyagorn, Wim Wuyts, and Athol U Wells.
• Louis Pradel Hospital, Reference Center for Rare Pulmonary Diseases, Hospices Civils de Lyon, UMR 754, Université Claude Bernard Lyon 1, Lyon, France.
• Eur Respir Rev. 2018 Dec 31; 27 (150).

AbstractAlthough these conditions are rare, a proportion of patients with interstitial lung diseases (ILDs) may develop a progressive-fibrosing phenotype. Progressive fibrosis is associated with worsening respiratory symptoms, lung function decline, limited response to immunomodulatory therapies, decreased quality of life and, potentially, early death. Idiopathic pulmonary fibrosis may be regarded as a model for other progressive-fibrosing ILDs. Here we focus on other ILDs that may present a progressive-fibrosing phenotype, namely idiopathic nonspecific interstitial pneumonia, unclassifiable idiopathic interstitial pneumonia, connective tissue disease-associated ILDs (e.g. rheumatoid arthritis-related ILD), fibrotic chronic hypersensitivity pneumonitis, fibrotic chronic sarcoidosis and ILDs related to other occupational exposures. Differential diagnosis of these ILDs can be challenging, and requires detailed consideration of clinical, radiological and histopathological features. Accurate and early diagnosis is crucial to ensure that patients are treated optimally.Copyright ©ERS 2018.

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