Clinical Course of Interstitial Lung Diseases: European IPF Registry and Biobank (eurIPFreg)

February 27, 2024 updated by: Andreas Guenther

The European IPF Registry - an Internet-based, Pan-European Registry Linked to the European IPF Biobank (eurIPFbank)

Born out of the European Union 7th Framework Programme funded project European IPF Network (eurIPFnet), the European IPF Registry (eurIPFreg) has become Europe's leading database of longitudinal data from IPF patients, including control groups of patients with other lung diseases. The registry was initiated with the intention of creating a permanent and continuously growing record of well defined data on IPF in Europe, in order to increase the chances of finding better treatment options for this devastating disease.

Clinical colleagues who would like to actively participate (both in terms of patient recruitment and data analysis) are invited to contact us (http://www.pulmonary-fibrosis.net/).

Study Overview

Detailed Description

The group's work aims to foster research on Idiopathic Pulmonary Fibrosis (IPF), the most aggressive form of an Idiopathic Interstitial Pneumonia (IIP). Within the eurIPFreg we, the eurIPFreg steering committee and a growing number of external site investigators, aim to describe the natural course of IPF and other IIPs, to identify risk factors that are associated with the evolution of the disease and to sample biomaterials that may serve as underlying basis for translational research activities.

IPF and non-specific interstitial pneumonia (NSIP), as well as the other entities of IIPs (cryptogenic organizing pneumonia, COP; desquamative interstitial pneumonia, DIP; respiratory bronchiolitis interstitial lung disease, RB-ILD; lymphoid interstitial pneumonia, LIP; acute interstitial pneumonia, AIP) are frequently progressive, fibroproliferative diseases of unknown etiology, affecting the lung parenchyma. Patients with IPF have the most devastating prognosis within the group of IIPs, with a median survival rate of 2-3 years.

Study Type

Observational

Enrollment (Estimated)

2000

Contacts and Locations

This section provides the contact details for those conducting the study, and information on where this study is being conducted.

Study Contact

Study Contact Backup

Study Locations

      • Vienna, Austria
        • Medizinische Universitat Wien
      • Dijon, France
        • Centre Hospitalier Universitaire Dijon
      • Paris, France
        • Hopital Bichat Paris
      • Giessen, Germany, 35392
        • Andreas Guenther
      • Greifenstein, Germany
        • Lungenfachklinik Waldhof Elgershausen
      • Catania, Italy
        • Università degli Studi di Catania
      • London, United Kingdom
        • Royal Brompton Hospital

Participation Criteria

Researchers look for people who fit a certain description, called eligibility criteria. Some examples of these criteria are a person's general health condition or prior treatments.

Eligibility Criteria

Ages Eligible for Study

16 years and older (Adult, Older Adult)

Accepts Healthy Volunteers

Yes

Sampling Method

Probability Sample

Study Population

Patients with Interstitial Lung Diseases

Description

Inclusion Criteria:

  • Informed consent signed

Exclusion Criteria:

  • No informed consent signed

Study Plan

This section provides details of the study plan, including how the study is designed and what the study is measuring.

How is the study designed?

Design Details

  • Observational Models: Cohort
  • Time Perspectives: Cross-Sectional

Cohorts and Interventions

Group / Cohort
Intervention / Treatment
Idiopathic Pulmonary Fibrosis (IPF)

IPF diagnosis according to the guidelines of 2011 (AJRCCM 2011; 183:788). For patients diagnosed prior to 2011, the criteria of the consensus statement 2000 apply (AJRCCM 2000;161:646).

Patient registry (observation and biomaterial sampling).

data are collected with patient questionnaires, additionally clinical data are collected at every routine visit and biomaterial is collected
Non-specific interstitial pneumonia

Non-specific interstitial pneumonia (NSIP) based on the histopathological demonstration of an NSIP pattern.

Patient registry (observation and biomaterial sampling).

data are collected with patient questionnaires, additionally clinical data are collected at every routine visit and biomaterial is collected
Cryptogenic organising pneumonia (COP)

COP characterised histologically by an organising pneumonia with intraluminal organising fibrosis in the alveolar ducts and alveolar spaces.

Patient registry (observation and biomaterial sampling).

data are collected with patient questionnaires, additionally clinical data are collected at every routine visit and biomaterial is collected
Acute interstitial pneumonia (AIP)

Histological pattern of diffuse alveolar damage (DAD), characterised by hyaline membranes, alveolar oedema and a marked interstitial and alveolar inflammatory reaction.

Patient registry (observation and biomaterial sampling).

data are collected with patient questionnaires, additionally clinical data are collected at every routine visit and biomaterial is collected
Lymphoid interstitial pneumonia (LIP)

Histological pattern of LIP primary or secondary (e.g. rheumatoid arthritis, Sjögren's syndrome, pernicious anaemia, chronic active hepatitis, systemic lupus erythematosus (SLE), primary biliary cirrhosis, myasthenia gravis, severe immune deficiency syndromes (AIDS)).

Patient registry (observation and biomaterial sampling).

data are collected with patient questionnaires, additionally clinical data are collected at every routine visit and biomaterial is collected
respiratory bronchiolitis-ILD (RB-ILD)
Histological pattern of RB-ILD or typical clinical and radiological findings. Patient registry (observation and biomaterial sampling).
data are collected with patient questionnaires, additionally clinical data are collected at every routine visit and biomaterial is collected
Desquamative Interstitial Pneumonia

Histological pattern of Desquamative Interstitial Pneumonia (DIP). The picture is similar to RB-ILD, but the distribution pattern is much more homogeneous and does not even have the bronchiolocentric distribution.

Patient registry (observation and biomaterial sampling).

data are collected with patient questionnaires, additionally clinical data are collected at every routine visit and biomaterial is collected
Hypersensitivity Pneumonitis

Hypersensitivity Pneumonitis (HP) characterized by exposure to inhaled organic antigens and development of antibodies. Typical clinical and radiological findings, lymphocytosis in bronchoalveolar lavage (BAL) or histology showing HP granulomas.

Patient registry (observation and biomaterial sampling).

data are collected with patient questionnaires, additionally clinical data are collected at every routine visit and biomaterial is collected
Sarcoidosis

Histological pattern with sarcoid granulomas or typical clinical and radiological findings with a lymphocytosis in BAL.

Patient registry (observation and biomaterial sampling).

data are collected with patient questionnaires, additionally clinical data are collected at every routine visit and biomaterial is collected
Lung Cancer

Histological confirmation of Lung Cancer. Patients will be included as control group.

Patient registry (observation and biomaterial sampling).

data are collected with patient questionnaires, additionally clinical data are collected at every routine visit and biomaterial is collected
Chronic Obstructive Pulmonary Disease

Obstructive spirometry and physical history suggesting Chronic Obstructive Pulmonary Disease (COPD). Patients will be included as control group.

Patient registry (observation and biomaterial sampling).

data are collected with patient questionnaires, additionally clinical data are collected at every routine visit and biomaterial is collected
Pulmonary Hypertension

Pulmonary Hypertension (PH) diagnosed through right heart catheterisation. Patients will be included as control group.

Patient registry (observation and biomaterial sampling).

data are collected with patient questionnaires, additionally clinical data are collected at every routine visit and biomaterial is collected
Sleep Apnea

Sleep Apnea diagnosed by polysomnography. Patients will be included as control group.

Patient registry (observation and biomaterial sampling).

data are collected with patient questionnaires, additionally clinical data are collected at every routine visit and biomaterial is collected
Asthma

Asthma diagnosed by positive bronchoprovocation test and typical history or bronchoreversibility in the lung function measurement or through peak flow measurement. Patients will be included as control group.

Patient registry (observation and biomaterial sampling).

data are collected with patient questionnaires, additionally clinical data are collected at every routine visit and biomaterial is collected
Control/Health Individuals
Healthy volunteers not suffering from any lung disease as control group. Patient registry (observation and biomaterial sampling).
data are collected with patient questionnaires, additionally clinical data are collected at every routine visit and biomaterial is collected

What is the study measuring?

Primary Outcome Measures

Outcome Measure
Measure Description
Time Frame
clinical course of patients with Interstitial Lung Diseases (ILD)
Time Frame: 5 years
change of lung function parameter such as forced vital capacity (FVC), diffusing lung capacity (DLCO) over time mortality symptoms (reported in patients questionnaires)
5 years

Secondary Outcome Measures

Outcome Measure
Measure Description
Time Frame
Comorbidities of patients with ILD
Time Frame: 5 years
reported in patients and physicians questionnaires
5 years
Infections in lung function of patients with ILD
Time Frame: 5 years
reported in patients and physicians questionnaires
5 years
Quality of life of patients with ILD
Time Frame: 5 years
reported in patients and physicians questionnaires, EQ5D (European quality of life 5-dimensions) questionnaire
5 years
Health care utilization of patients with ILD
Time Frame: 5 years
reported in patients questionnaires
5 years

Collaborators and Investigators

This is where you will find people and organizations involved with this study.

Publications and helpful links

The person responsible for entering information about the study voluntarily provides these publications. These may be about anything related to the study.

General Publications

Study record dates

These dates track the progress of study record and summary results submissions to ClinicalTrials.gov. Study records and reported results are reviewed by the National Library of Medicine (NLM) to make sure they meet specific quality control standards before being posted on the public website.

Study Major Dates

Study Start

September 1, 2009

Primary Completion (Estimated)

January 1, 2025

Study Completion (Estimated)

January 1, 2040

Study Registration Dates

First Submitted

June 9, 2015

First Submitted That Met QC Criteria

October 28, 2016

First Posted (Estimated)

November 1, 2016

Study Record Updates

Last Update Posted (Actual)

February 28, 2024

Last Update Submitted That Met QC Criteria

February 27, 2024

Last Verified

February 1, 2024

More Information

Terms related to this study

Plan for Individual participant data (IPD)

Plan to Share Individual Participant Data (IPD)?

UNDECIDED

This information was retrieved directly from the website clinicaltrials.gov without any changes. If you have any requests to change, remove or update your study details, please contact register@clinicaltrials.gov. As soon as a change is implemented on clinicaltrials.gov, this will be updated automatically on our website as well.

Clinical Trials on Idiopathic Pulmonary Fibrosis

Clinical Trials on patient registry (observation and biomaterial sampling)

3
Subscribe