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

• Sponsor: Andreas Guenther
• Information provided by (Responsible Party): Andreas Guenther, University of Giessen

Study Description

Brief Summary:

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/).

Condition or disease	Intervention/treatment
Idiopathic Pulmonary Fibrosis; Idiopathic Interstitial Pneumonia; Interstitial Lung Diseases; Diffuse Parenchymal Lung Diseases	Other: patient registry (observation and biomaterial sampling)

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 Design

Layout table for study information

Study Type :	Observational [Patient Registry]
Estimated Enrollment :	2000 participants
Observational Model:	Cohort
Time Perspective:	Cross-Sectional
Target Follow-Up Duration:	60 Months
Official Title:	The European IPF Registry - an Internet-based, Pan-European Registry Linked to the European IPF Biobank (eurIPFbank)
Study Start Date :	September 2009
Estimated Primary Completion Date :	January 2025
Estimated Study Completion Date :	January 2040

Groups and Cohorts

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).	Other: 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).	Other: 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).	Other: 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).	Other: 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).	Other: 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).	Other: 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).	Other: 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).	Other: 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).	Other: 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).	Other: 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).	Other: 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).	Other: 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).	Other: 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).	Other: 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).	Other: 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

Outcome Measures

Primary Outcome Measures :

1. 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)

Secondary Outcome Measures :

1. Comorbidities of patients with ILD [ Time Frame: 5 years ]
   reported in patients and physicians questionnaires
2. Infections in lung function of patients with ILD [ Time Frame: 5 years ]
   reported in patients and physicians questionnaires
3. 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
4. Health care utilization of patients with ILD [ Time Frame: 5 years ]
   reported in patients questionnaires

Biospecimen Retention:   Samples With DNA

EDTA blood, Plasma, Serum, GenePAX blood, BALF, BALCP, lung tissue

Eligibility Criteria

Layout table for eligibility information

Ages Eligible for Study:	18 Years and older (Adult, Older Adult)
Sexes Eligible for Study:	All
Accepts Healthy Volunteers:	Yes
Sampling Method:	Probability Sample

Study Population

Patients with Interstitial Lung Diseases

Criteria

Inclusion Criteria:

• Informed consent signed

Exclusion Criteria:

• No informed consent signed

Contacts and Locations

Contacts

Layout table for location contacts

Contact: Andreas Guenther, MD	+49 64198542502	andreas.guenther@innere.med.uni-giessen.de
Contact: Jasmin Wagner, MD	+49 64198542502	jasmin.wagner@innere.med.uni-giessen.de

Locations

Layout table for location information

Austria
Medizinische Universität Wien	Recruiting
Vienna, Austria
Contact: Walter Klepetko
France
Centre Hospitalier Universitaire Dijon	Recruiting
Dijon, France
Contact: Philippe Bonniaud
Hopital Bichat Paris	Recruiting
Paris, France
Contact: Bruno Crestani
Germany
Andreas Guenther	Recruiting
Giessen, Germany, 35392
Contact: Andreas Guenther, MD 64198542502 ext 502 andreas.guenther@innere.med.uni-giessen.de
Principal Investigator: Andreas Guenther, MD
Sub-Investigator: Daniel von der Beck, MD
Sub-Investigator: Fotios Drakopanagiotakis, MD
Lungenfachklinik Waldhof Elgershausen	Recruiting
Greifenstein, Germany
Contact: Andreas Guenther, MD +49 6449 927 ext 262
Principal Investigator: Andreas Guenther, MD
Sub-Investigator: Benjamin Loeh, MD
Italy
Università degli Studi di Catania	Recruiting
Catania, Italy
Contact: Carlo Vancheri
United Kingdom
Royal Brompton Hospital	Recruiting
London, United Kingdom
Contact: Atholl Wells

Sponsors and Collaborators

Andreas Guenther

More Information

Additional Information:

European IPF Registry website 

Publications:

Guenther A; European IPF Network. The European IPF Network: towards better care for a dreadful disease. Eur Respir J. 2011 Apr;37(4):747-8. doi: 10.1183/09031936.00012111.

Loeh B, Drakopanagiotakis F, Bandelli GP, von der Beck D, Tello S, Cordani E, Rizza E, Barrocu L, Markart P, Seeger W, Guenther A, Albera C. Intraindividual response to treatment with pirfenidone in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2015 Jan 1;191(1):110-3. doi: 10.1164/rccm.201406-1106LE.

Publications automatically indexed to this study by ClinicalTrials.gov Identifier (NCT Number):

Witt S, Krauss E, Barbero MAN, Müller V, Bonniaud P, Vancheri C, Wells AU, Vasakova M, Pesci A, Klepetko W, Seeger W, Crestani B, Leidl R, Holle R, Schwarzkopf L, Guenther A. Psychometric properties and minimal important differences of SF-36 in Idiopathic Pulmonary Fibrosis. Respir Res. 2019 Mar 1;20(1):47. doi: 10.1186/s12931-019-1010-5.

Loeh B, Brylski LT, von der Beck D, Seeger W, Krauss E, Bonniaud P, Crestani B, Vancheri C, Wells AU, Markart P, Breithecker A, Guenther A. Lung CT Densitometry in Idiopathic Pulmonary Fibrosis for the Prediction of Natural Course, Severity, and Mortality. Chest. 2019 Feb 8. pii: S0012-3692(19)30128-X. doi: 10.1016/j.chest.2019.01.019. [Epub ahead of print]

Guenther A, Krauss E, Tello S, Wagner J, Paul B, Kuhn S, Maurer O, Heinemann S, Costabel U, Barbero MAN, Müller V, Bonniaud P, Vancheri C, Wells A, Vasakova M, Pesci A, Sofia M, Klepetko W, Seeger W, Drakopanagiotakis F, Crestani B. The European IPF registry (eurIPFreg): baseline characteristics and survival of patients with idiopathic pulmonary fibrosis. Respir Res. 2018 Jul 28;19(1):141. doi: 10.1186/s12931-018-0845-5.

Layout table for additonal information

Responsible Party:	Andreas Guenther, Professor of Internal Medicine, University of Giessen
ClinicalTrials.gov Identifier:	NCT02951416 History of Changes
Other Study ID Numbers:	EurIPFreg_150609
First Posted:	November 1, 2016
Last Update Posted:	November 20, 2017
Last Verified:	November 2017
Individual Participant Data (IPD) Sharing Statement:
Plan to Share IPD:	Undecided

Additional relevant MeSH terms:

Layout table for MeSH terms

Pneumonia; Lung Diseases; Pulmonary Fibrosis; Idiopathic Pulmonary Fibrosis	Idiopathic Interstitial Pneumonias; Lung Diseases, Interstitial; Respiratory Tract Diseases; Respiratory Tract Infections