{
  "abstract": "Background Prior work suggests different interstitial lung diseases (ILDs) that share the radiological usual interstitial pneumonia (UIP) pattern have an overall worse prognosis. However, epidemiological data with longitudinal sampling and replication remains lacking.Methods Data was used from the Pulmonary Fibrosis Foundation Patient Registry (PFF-PR) (n=932) and a meta-cohort of ILD research studies (n=1579). Linear mixed-effects models and Cox proportional hazard models were used to determine forced vital capacity (FVC) slopes and 5-year transplant-free survival, respectively, by ILD diagnosis and UIP radiological pattern. Secondarily, we examined FVC and survival by diagnosis and radiological fibrosis quantified by data-driven texture analysis (DTA) in the PFF-PR. Models were adjusted for age, sex, smoking and antifibrotic and immunosuppression medication use.Results The proportions of idiopathic pulmonary fibrosis (IPF), fibrotic hypersensitivity pneumonitis (FHP) and connective tissue disease (CTD)-ILD were the following for PFF-PR (70%, 11%, 19%) and meta-cohort (21%, 32%, 47%). In the PFF-PR, CTD-ILD with UIP CT pattern was associated with slower FVC decline (−34.4 mL/year) compared with IPF (−158.4 mL/year) and longer transplant-free survival (HR 0.50, 95% CI 0.29 to 0.85). This was replicated in the meta cohort for FVC (−53.1 vs −185.9 mL/year, p<0.0001) and survival (HR 0.38, 95% CI 0.27 to 0.53). A similar pattern was seen using DTA to objectively categorise patients into higher and lower radiological fibrosis. Between IPF and FHP-UIP, FVC decline was not significantly different in the PFF-PR (−203.4 vs −158.4 mL/year, p=0.58) and meta-cohort (−124.0 vs −185.9 mL/year, p=0.25).Conclusions Even in the presence of a UIP CT pattern, there may still be differences in lung function over time and survival, particularly for CTD-ILD.",
  "authors": [
    {
      "affiliations": [
        "Department of Medicine, University of Virginia School of Medicine, Charlottesville, Virginia, USA"
      ],
      "name": "John S Kim"
    },
    {
      "affiliations": [
        "Department of Medicine, University of Michigan, Ann Arbor, Michigan, USA"
      ],
      "name": "Janelle Pugashetti"
    },
    {
      "affiliations": [
        "Department of Medicine, University of Virginia School of Medicine, Charlottesville, Virginia, USA"
      ],
      "name": "Shwu-Fan Ma"
    },
    {
      "affiliations": [
        "Department of Medicine, University of Virginia School of Medicine, Charlottesville, Virginia, USA"
      ],
      "name": "Yong Huang"
    },
    {
      "affiliations": [
        "Department of Medicine, Weill Cornell Medical College, New York, New York, USA"
      ],
      "name": "Anna J Podolanczuk"
    },
    {
      "affiliations": [
        "Radiology, National Jewish Health, Denver, Colorado, USA"
      ],
      "name": "David A Lynch"
    },
    {
      "affiliations": [
        "Department of Radiology, University of California Los Angeles, Los Angeles, California, USA"
      ],
      "name": "Andrea Oh"
    },
    {
      "affiliations": [
        "Department of Genome Sciences, University of Virginia School of Medicine, Charlottesville, Virginia, USA"
      ],
      "name": "Josyf Mychaleckyj"
    },
    {
      "affiliations": [
        "Department of Genome Sciences, University of Virginia School of Medicine, Charlottesville, Virginia, USA"
      ],
      "name": "Ani Manichaikul"
    },
    {
      "affiliations": [
        "Department of Genome Sciences, University of Virginia School of Medicine, Charlottesville, Virginia, USA"
      ],
      "name": "Stephen Rich"
    },
    {
      "affiliations": [
        "Department of Respiratory Medicine, Royal Brompton Hospital, Guy’s and St. Thomas’s NHS Foundation Trust and National Heart and Lung Institute Imperial College London, London, UK"
      ],
      "name": "Felix Chua"
    },
    {
      "affiliations": [
        "Department of Medicine, University of Texas Southwestern Medical Center, Dallas, Texas, USA"
      ],
      "name": "Traci M Adams"
    },
    {
      "affiliations": [
        "Department of Medicine, University of Michigan, Ann Arbor, Michigan, USA"
      ],
      "name": "Kevin R Flaherty"
    },
    {
      "affiliations": [
        "Department of Medicine, University of Colorado, Denver, Colorado, USA"
      ],
      "name": "Joyce S Lee"
    },
    {
      "affiliations": [
        "Medicine, Tulane University, New Olreans, Louisiana, USA"
      ],
      "name": "Joseph A Lasky"
    },
    {
      "affiliations": [
        "University of Washington, Seattle, Washington, USA"
      ],
      "name": "Ganesh Raghu"
    },
    {
      "affiliations": [
        "Biostatistics, University of Michigan, Ann Arbor, Michigan, USA"
      ],
      "name": "Susan Murray"
    },
    {
      "affiliations": [
        "Biostatistics, University of Michigan, Ann Arbor, Michigan, USA"
      ],
      "name": "Catherine Spino"
    },
    {
      "affiliations": [
        "Department of Medicine, University of Massachusetts Chan Medical School-UMass Memorial Health System, Worcester, Massachusetts, USA"
      ],
      "name": "Fernando J Martinez"
    },
    {
      "affiliations": [
        "Department of Medicine, University of Virginia School of Medicine, Charlottesville, Virginia, USA"
      ],
      "name": "Imre Noth"
    },
    {
      "affiliations": [
        "Radiology, National Jewish Health, Denver, Colorado, USA"
      ],
      "name": "Stephen M Humphries"
    },
    {
      "affiliations": [
        "Pulmonary and Critical Care Medicine, University of Chicago, Chicago, Illinois, USA"
      ],
      "name": "Ayodeji Adegunsoye"
    },
    {
      "affiliations": [
        "Department of Respiratory Medicine, Royal Brompton Hospital, Guy’s and St. Thomas’s NHS Foundation Trust and National Heart and Lung Institute Imperial College London, London, UK"
      ],
      "name": "Philip L Molyneaux"
    },
    {
      "affiliations": [
        "Department of Medicine, University of Michigan, Ann Arbor, Michigan, USA"
      ],
      "name": "Justin M Oldham"
    },
    {
      "affiliations": [
        "Department of Medicine, University of Texas Southwestern Medical Center, Dallas, Texas, USA"
      ],
      "name": "Chad A Newton"
    }
  ],
  "title": "Associations of interstitial lung disease subtype and CT pattern with lung function and survival",
  "uid": "d50b3c3a-128e-58a6-8f0e-c87a6a964580"
}