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Clinical characteristics of patients with familial idiopathic pulmonary fibrosis (f-IPF).
- Source :
-
BMC pulmonary medicine [BMC Pulm Med] 2019 Jul 18; Vol. 19 (1), pp. 130. Date of Electronic Publication: 2019 Jul 18. - Publication Year :
- 2019
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Abstract
- Background: The aim of this study was to analyze the relative frequency, clinical characteristics, disease onset and progression in f-IPF vs. sporadic IPF (s-IPF).<br />Methods: Familial 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.<br />Results: Within 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).<br />Conclusions: The 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.<br />Trial Registration: Nr. NCT02951416 http://www.www.clinicaltrials.gov.
- Subjects :
- Aged
Cross-Sectional Studies
Disease Progression
Europe epidemiology
Female
Humans
Idiopathic Interstitial Pneumonias mortality
Idiopathic Pulmonary Fibrosis mortality
Idiopathic Pulmonary Fibrosis physiopathology
Lung physiopathology
Male
Middle Aged
Survival Rate
Idiopathic Interstitial Pneumonias diagnosis
Idiopathic Pulmonary Fibrosis diagnosis
Registries
Subjects
Details
- Language :
- English
- ISSN :
- 1471-2466
- Volume :
- 19
- Issue :
- 1
- Database :
- MEDLINE
- Journal :
- BMC pulmonary medicine
- Publication Type :
- Academic Journal
- Accession number :
- 31319833
- Full Text :
- https://doi.org/10.1186/s12890-019-0895-6