Dataset Information

Idiopathic pulmonary fibrosis patients with severe physiologic impairment: characteristics and outcomes.

ABSTRACT:

Research question

There is no widely accepted grading system for IPF disease severity, although physiologic impairment based on pulmonary function testing is frequently employed. We sought to describe clinical and functional characteristics as well as outcomes of patients with severe physiologic impairment.

Patients and methods

IPF patients with severe physiologic impairment defined by FVC???50% and/or DLco???30% predicted evaluated in the Inova Advanced Lung Disease Program between 2011 and 2019 were included. Demographic, physiologic, functional treatment and outcome data were collated.

Results

There were 531 patients with IPF evaluated of whom 242 (46%) had severe physiologic impairment. Mean age was 72?±?8 years; baseline FVC was 53?±?17% and DL_CO 28?±?9% of predicted. The mean 6 min walks test (6MWT) distance was 304?±?121 m with 59% of the patients requiring supplemental oxygen ([Formula: see text] group). There was a poor correlation between the 6MWT distance and both FVC% and DLco%. Patients in the 6MWT_RA group had a better transplant-free survival than the [Formula: see text] group (p?=?0.002). Patients managed before October 2014 and not receiving antifibrotic therapy had worse outcomes with reduced transplant-free survival compared with patients presenting after this date who did receive antifibrotic therapy (n?=?113) (log rank p?ConclusionIPF patients often present with severe physiologic impairment which may be poorly correlated with their functional status. Assessment of IPF disease severity should not be based on physiologic impairment alone, but should also encompass functional status as well as need for supplemental oxygen. Antifibrotic therapy in patients with severe physiologic impairment is associated with improved outcomes.

SUBMITTER: Pastre J

PROVIDER: S-EPMC7788925 | biostudies-literature | 2021 Jan

REPOSITORIES: biostudies-literature

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Publications

Idiopathic pulmonary fibrosis patients with severe physiologic impairment: characteristics and outcomes.

Pastre Jean J Barnett Scott S Ksovreli Inga I Taylor Jeannie J Brown A Whitney AW Shlobin Oksana A OA Ahmad Kareem K Khangoora Vikramjit V Aryal Shambhu S King Christopher S CS Nathan Steven D SD

Respiratory research 20210106 1

<h4>Research question</h4>There is no widely accepted grading system for IPF disease severity, although physiologic impairment based on pulmonary function testing is frequently employed. We sought to describe clinical and functional characteristics as well as outcomes of patients with severe physiologic impairment.<h4>Patients and methods</h4>IPF patients with severe physiologic impairment defined by FVC ≤ 50% and/or DLco ≤ 30% predicted evaluated in the Inova Advanced Lung Disease Program betwe ...[more]

PMID: 33407450

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Project description:BackgroundThe aim of this study was to analyze the relative frequency, clinical characteristics, disease onset and progression in f-IPF vs. sporadic IPF (s-IPF).MethodsFamilial IPF index patients and their family members were recruited into the European IPF registry/biobank (eurIPFreg) at the Universities of Giessen and Marburg (UGMLC). Initially, we employed wide range criteria of f-IPF (e.g. relatives who presumably died of some kind of parenchymal lung disease). After narrowing down the search to occurrence of idiopathic interstitial pneumonia (IIP) in at least one first grade relative, 28 index patients were finally identified, prospectively interviewed and examined. Their family members were phenotyped with establishment of pedigree charts.ResultsWithin the 28 IPF families, overall 79 patients with f-IPF were identified. In the same observation period, 286 f-IIP and s-IIP patients were recruited into the eurIPFreg at our UGMLC sites, corresponding to a familial versus s-IPF of 9.8%. The both groups showed no difference in demographics (61 vs. 79% males), smoking history, and exposure to any environmental triggers known to cause lung fibrosis. The f-IPF group differed by an earlier age at the onset of the disease (55.4 vs. 63.2 years; p < 0.001). On average, the f-IPF patients presented a significantly milder extent of functional impairment at the time point of inclusion vs. the s-IPF group (FVC 75% pred. vs. FVC 62% pred., p = 0.011). In contrast, the decline in FVC was found to be faster in the f-IPF vs. the s-IPF group (4.94% decline in 6 months in f-IPF vs. 2.48% in s-IPF, p = 0.12). The average age of death in f-IPF group was 67 years vs. 71.8 years in s-IPF group (p = 0.059). The f-IIP group displayed diverse inheritance patterns, mostly autosomal-dominant with variable penetrance. In the f-IPF, the younger generations showed a tendency for earlier manifestation of IPF vs. the older generation (58 vs. 66 years, p = 0.013).ConclusionsThe 28 f-IPF index patients presented an earlier onset and more aggressive natural course of the disease. The disease seems to affect consecutive generations at a younger age.Trial registrationNr. NCT02951416 http://www.www.clinicaltrials.gov.

Dataset Information

Idiopathic pulmonary fibrosis patients with severe physiologic impairment: characteristics and outcomes.

Research question

Patients and methods

Results

Publications

Idiopathic pulmonary fibrosis patients with severe physiologic impairment: characteristics and outcomes.

Similar Datasets

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