Pulmonary Fibrosis and Telomerase Mutation Study

Groupe d'Etudes et de Recherche sur les Maladies Orphelines Pulmonaires

Status: Completed

Conditions: Pulmonary Fibrosis

Study type: Observational

Funder types: Other

Identifiers

NCT01501578

GERMOP-004

Details and patient eligibility

About

This study is an observational and retrospective study of patients with pulmonary fibrosis associated or not with telomerase mutation.

The purpose of this study is to describe in detail the cases with telomerase mutation in terms of features on CT scan, respiratory function and evolution, in comparison to control subjects with idiopathic pulmonary fibrosis and no telomerase mutation identified or family history.

Full description

Two "control" subjects will be enrolled for one subject with telomerase mutation.

The data are all the results of investigations conducted for the diagnosis of idiopathic pulmonary fibrosis and during routine follow up of patients.

The CT scans will reviewed centrally to homogenize the description.

Enrollment: 81 patients

Sex: All

Ages: 18+ years old

Volunteers: No Healthy Volunteers

Inclusion criteria

• Diffuse interstitial lung disease on CT scan
• Telomerase mutation analysis

Exclusion criteria

• Presence of connective tissue disease, or pneumoconiosis or drug induced lung disease

Trial design

81 participants in 2 patient groups

telomerase mutation

Description:

Patients with interstitial lung disease and telomerase mutation

control

Description:

Patients with idiopathic pulmonary fibrosis and without telomerase mutation