Presentation, diagnosis and clinical course of the spectrum of progressive-fibrosing interstitial lung diseases

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, Athol U Wells, 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, Athol U Wells

Abstract

Although 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.

Conflict of interest statement

Conflict of interest: V. Cottin reports receiving the following, outside the submitted work: personal fees from Actelion for consultancy, lectures and travel to medical meetings; personal fees from Boehringer Ingelheim for the development of educational presentations, consultancy, lectures and travel to medical meetings; personal fees from Bayer for consultancy; personal fees from Gilead for acting as a member of an adjudication committee; personal fees from GSK for consultancy; personal fees from MSD for consultancy and travel to medical meetings; personal fees from Novartis for consultancy and lectures; personal fees from Roche for consultancy, lecture fees and travel to medical meetings; personal fees from Sanofi for consultancy and lectures; a grant to his institution from Boehringer Ingelheim; a grant to his institution from Roche; personal fees from Promedior for acting as Chair of the DSMB; personal fees from Celgene for the DSMB; and personal fees from Galapagos for consultancy and for acting as Chair of the DSMB. Conflict of interest: N.A. Hirani has nothing to disclose. Conflict of interest: D.L. Hotchkin has nothing to disclose. Conflict of interest: A.M. Nambiar reports receiving the following, outside the submitted work: grants, personal fees, non-financial support and other support from Boehringer Ingelheim; and grants from Genentech-Roche. Conflict of interest: T. Ogura reports receiving the following, outside the submitted work: grants and personal fees from Boehringer Ingelheim, Japan; grants from the Ministry of Health, Labour and Welfare, Japan; personal fees from Astellas Pharma Inc., Shionogi & Co. Ltd, Toray Industries Inc., AstraZeneca K.K. and Kyorin Inc. Conflict of interest: M. Otaola has nothing to disclose. Conflict of interest: D. Skowasch reports receiving the following, outside the submitted work: personal fees/honoraria for consulting and speaking from Boehringer Ingelheim and Roche. Conflict of interest: J.S. Park has nothing to disclose. Conflict of interest: H.K. Poonyagariyagorn has nothing to disclose. Conflict of interest: W. Wuyts reports receiving the following, outside the submitted work: grants paid to his university from Boehringer Ingelheim and Hoffmann La Roche; and travel fees from Galapagos. Conflict of interest: A.U. Wells reports receiving the following, outside the submitted work: personal fees for speaking and for acting on advisory boards from Boehringer Ingelheim, Roche and Bayer.

Copyright ©ERS 2018.

Figures

FIGURE 1
FIGURE 1
Types of interstitial lung disease (ILD) most likely to have a progressive-fibrosing phenotype (indicated in bold). IIPs: idiopathic interstitial pneumonias. #: stage IV sarcoidosis only; ¶: not an established clinical diagnosis; +: e.g. asbestosis, silicosis.
FIGURE 2
FIGURE 2
Diagnosis of fibrosing interstitial lung diseases (ILD) that may present a progressive phenotype. PFTs: pulmonary function test; HCRT: high-resolution computed tomography; BAL: bronchoalveolar lavage; MDD: multidisciplinary diagnosis; PF-ILD: progressive-fibrosing ILD.
FIGURE 3
FIGURE 3
Nonspecific interstitial pneumonia: high-resolution computed tomography images from a 46-year-old male patient who underwent lung transplantation. a) The initial scan, taken at first admission, shows ground-glass attenuation and consolidation with reticulation and traction bronchiectasis along with a bronchovascular bundle, sparing the subpleural lung. b) A follow-up scan 3 years later showed increased ground-glass opacity and consolidation despite corticosteroids and immunosuppressive therapy. c) After another 3 years, a decrease in ground-glass opacity and consolidation was evident, together with increased traction bronchiectasis and cysts.

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