AI Article Synopsis

  • The study focuses on the prevalence and prognosis of clinically progressive fibrosis in interstitial lung diseases (ILDs), particularly comparing idiopathic pulmonary fibrosis (IPF) and non-IPF fibrosing ILD (FILD) patients.
  • Out of 844 evaluated patients, 42.1% showed signs of progressive fibrosis, with 59.4% of IPF patients and 26.6% of non-IPF FILD patients meeting the criteria.
  • The research found that having a progressive phenotype significantly affects transplantation-free survival, but the survival rates for non-IPF FILD patients with this phenotype are comparable to those of IPF patients.

Article Abstract

Background And Objective: The development of clinically progressive fibrosis complicates a wide array of interstitial lung diseases (ILDs). However, there are limited data regarding its prevalence and prognosis.

Methods: We analysed consecutive patients seen for initial evaluation of a fibrosing form of ILD (FILD). Patients were evaluated for evidence of progressive fibrosis over the first 24 months of follow-up. We defined a progressive phenotype as the presence of at least one of the following: a relative decline in forced vital capacity (FVC) of ≥10%; a relative decline in FVC of ≥5%-<10% with a relative decline in diffusing capacity of the lung for carbon monoxide of ≥15%, increased fibrosis on HRCT or progressive symptoms.

Results: Eight hundred and forty-four patients (397 with idiopathic pulmonary fibrosis [IPF] and 447 non-IPF FILD) made up the final analysis cohort. Three hundred and fifty-five patients (42.1%) met the progressive phenotype criteria (59.4% of IPF patients and 26.6% of non-IPF FILD patients, p <0.01). In both IPF and non-IPF FILD, transplantation-free survival differed between patients with a progressive phenotype and those without (p <0.01). Multivariable analysis showed that a progressive phenotype was an independent predictor of transplantation-free survival (hazard ratio [HR]: 3.36, 95% CI: 2.68-4.23, p <0.01). Transplantation-free survival did not differ between non-IPF FILD with a progressive phenotype and IPF (HR: 1.12, 95% CI: 0.85-1.48, p = 0.42).

Conclusion: Over one-fourth of non-IPF FILD patients develop a progressive phenotype compared to approximately 60% of IPF patients. The survival of non-IPF FILD patients with a progressive phenotype is similar to IPF.

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Source
http://dx.doi.org/10.1111/resp.14245DOI Listing

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