Progressive Fibrosing Interstitial Lung Diseases: Prevalence and Characterization in Two Italian Referral Centers

P. Faverio, M. Piluso, F. De Giacomi, M. Della Zoppa, R. Cassandro, S. Harari, F. Luppi, A. Pesci

Research output: Contribution to journalArticlepeer-review


Background: The prevalence and natural history of progressive fibrosing interstitial lung diseases (PF-ILDs), and their response to commonly used treatments in real life are largely unknown. Objectives: The aim of the study was to describe the prevalence, clinical characteristics, management, and outcomes of PF-ILD patients attending 2 Italian referral centers (San Gerardo Hospital, Monza, and San Giuseppe Hospital, Milan) from January 1, 2011, to July 31, 2019. Methods: From a cohort of non-idiopathic pulmonary fibrosis fibrosing ILD patients with at least 2-year follow-up, we selected only those with progressive disease, defined as per the INBUILD trial, collecting their demographical, clinical, and functional data. Results: Out of the 245 fibrosing ILD patients, 75 (31%) were classified as PF-ILDs (median age 66 years, 60% males), most frequently idiopathic non-specific interstitial pneumonia (28%), followed by connective tissue disease-associated ILD (20%), chronic hypersensitivity pneumonitis, and sarcoidosis (17% each). Most patients (81%) were categorized as PF-ILDs because of forced vital capacity (FVC) decline ≥10%, while 19% experienced a marginal FVC decline (between 5 and 10%) associated with worsening respiratory symptoms or increasing extent of fibrotic changes on high-resolution computed tomography. Disease progression occurred after a median of 18 months from ILD diagnosis. The vast majority (93%) of PF-ILD patients received prednisolone, alone (40%) or associated with steroid-sparing agents (52%), and 35% of treated patients developed treatment-related adverse events. After ILD progression, the median survival was 3 (interquartile range (IQR) 2-5) years, with a 2-and 3-year mortality rate of 4 and 20%, respectively. Conclusions: In a real-life setting, approximately one-third of the fibrosing ILD patients showed a progressive course despite treatment. Studies aimed to better phenotype this subgroup of patients are needed. © 2020 S. Karger AG, Basel. Copyright: All rights reserved.
Original languageEnglish
Pages (from-to)838-845
Number of pages8
Issue number10
Publication statusPublished - 2021


  • Anti-fibrotic agents
  • Interstitial lung diseases
  • Mortality
  • Progressive fibrosing
  • Treatment
  • azathioprine
  • cyclophosphamide
  • cyclosporine
  • hydroxychloroquine
  • methotrexate
  • mycophenolate mofetil
  • prednisone
  • rituximab
  • adult
  • aged
  • aging
  • allergic pneumonitis
  • Article
  • body weight gain
  • bone marrow disease
  • cataract
  • clinical study
  • cohort analysis
  • connective tissue disease
  • demography
  • diabetes mellitus
  • disease association
  • disease course
  • drug safety
  • female
  • fibrosing alveolitis
  • follow up
  • forced vital capacity
  • gastrointestinal symptom
  • health care management
  • high resolution computer tomography
  • human
  • Italy
  • liver toxicity
  • major clinical study
  • male
  • median survival time
  • mortality rate
  • osteoporosis
  • prevalence
  • priority journal
  • psychosis
  • recurrent infection
  • sarcoidosis
  • sex ratio
  • side effect
  • treatment outcome

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