Clinical characteristics of patients with familial idiopathic pulmonary fibrosis (f-IPF).

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).

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.

Results

Within the

SUBMITTER: Krauss E 

PROVIDER: S-EPMC6637501 | biostudies-literature | 2019 Jul

REPOSITORIES: biostudies-literature