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

NCT ID: NCT02951416

Last Updated: 2024-02-28

Study Results

Results pending

The study team has not published outcome measurements, participant flow, or safety data for this trial yet. Check back later for updates.

Basic Information

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Recruitment Status

ACTIVE_NOT_RECRUITING

Total Enrollment

2000 participants

Study Classification

OBSERVATIONAL

Study Start Date

2009-09-30

Study Completion Date

2040-01-31

Brief Summary

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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/).

Detailed Description

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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.

Conditions

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Idiopathic Pulmonary Fibrosis Idiopathic Interstitial Pneumonia Interstitial Lung Diseases Diffuse Parenchymal Lung Diseases

Study Design

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Observational Model Type

COHORT

Study Time Perspective

CROSS_SECTIONAL

Study Groups

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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).

patient registry (observation and biomaterial sampling)

Intervention Type OTHER

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).

patient registry (observation and biomaterial sampling)

Intervention Type OTHER

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).

patient registry (observation and biomaterial sampling)

Intervention Type OTHER

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).

patient registry (observation and biomaterial sampling)

Intervention Type OTHER

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).

patient registry (observation and biomaterial sampling)

Intervention Type OTHER

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).

patient registry (observation and biomaterial sampling)

Intervention Type OTHER

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).

patient registry (observation and biomaterial sampling)

Intervention Type OTHER

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).

patient registry (observation and biomaterial sampling)

Intervention Type OTHER

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).

patient registry (observation and biomaterial sampling)

Intervention Type OTHER

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).

patient registry (observation and biomaterial sampling)

Intervention Type OTHER

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).

patient registry (observation and biomaterial sampling)

Intervention Type OTHER

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).

patient registry (observation and biomaterial sampling)

Intervention Type OTHER

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).

patient registry (observation and biomaterial sampling)

Intervention Type OTHER

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).

patient registry (observation and biomaterial sampling)

Intervention Type OTHER

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).

patient registry (observation and biomaterial sampling)

Intervention Type OTHER

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

Interventions

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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

Intervention Type OTHER

Eligibility Criteria

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Inclusion Criteria

* Informed consent signed

Exclusion Criteria

* No informed consent signed
Minimum Eligible Age

18 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

Yes

Sponsors

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Andreas Guenther

OTHER

Sponsor Role lead

Responsible Party

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Andreas Guenther

Professor of Internal Medicine

Responsibility Role SPONSOR_INVESTIGATOR

Locations

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Medizinische Universität Wien

Vienna, , Austria

Site Status

Centre Hospitalier Universitaire Dijon

Dijon, , France

Site Status

Hopital Bichat Paris

Paris, , France

Site Status

Andreas Guenther

Giessen, , Germany

Site Status

Lungenfachklinik Waldhof Elgershausen

Greifenstein, , Germany

Site Status

Università degli Studi di Catania

Catania, , Italy

Site Status

Royal Brompton Hospital

London, , United Kingdom

Site Status

Countries

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Austria France Germany Italy United Kingdom

References

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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. No abstract available.

Reference Type BACKGROUND
PMID: 21454892 (View on PubMed)

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. No abstract available.

Reference Type BACKGROUND
PMID: 25551350 (View on PubMed)

Krauss E, Claas LH, Tello S, Naumann J, Wobisch S, Kuhn S, Majeed RW, Moor K, Molina-Molina M, Byrne O, Borton R, Wijsenbeek MS, Hirani N, Vancheri C, Crestani B, Guenther A; eurILDreg investigators. European ILD registry algorithm for self-assessment in interstitial lung diseases (eurILDreg ASA-ILD). PLoS One. 2025 Jan 29;20(1):e0316484. doi: 10.1371/journal.pone.0316484. eCollection 2025.

Reference Type DERIVED
PMID: 39879227 (View on PubMed)

Krauss E, Tello S, Naumann J, Wobisch S, Ruppert C, Kuhn S, Mahavadi P, Majeed RW, Bonniaud P, Molina-Molina M, Wells A, Hirani N, Vancheri C, Walsh S, Griese M, Crestani B, Guenther A; further eurILDreg investigators; RARE-ILD investigators. Protocol and research program of the European registry and biobank for interstitial lung diseases (eurILDreg). BMC Pulm Med. 2024 Nov 18;24(1):572. doi: 10.1186/s12890-024-03389-9.

Reference Type DERIVED
PMID: 39558302 (View on PubMed)

Krauss E, Haberer J, Barreto G, Degen M, Seeger W, Guenther A. Recognition of breathprints of lung cancer and chronic obstructive pulmonary disease using the Aeonose(R) electronic nose. J Breath Res. 2020 Jul 24;14(4):046004. doi: 10.1088/1752-7163/ab8c50.

Reference Type DERIVED
PMID: 32325432 (View on PubMed)

Krauss E, Gehrken G, Drakopanagiotakis F, Tello S, Dartsch RC, Maurer O, Windhorst A, von der Beck D, Griese M, Seeger W, Guenther A. Clinical characteristics of patients with familial idiopathic pulmonary fibrosis (f-IPF). BMC Pulm Med. 2019 Jul 18;19(1):130. doi: 10.1186/s12890-019-0895-6.

Reference Type DERIVED
PMID: 31319833 (View on PubMed)

Witt S, Krauss E, Barbero MAN, Muller 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.

Reference Type DERIVED
PMID: 30823880 (View on PubMed)

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 May;155(5):972-981. doi: 10.1016/j.chest.2019.01.019. Epub 2019 Feb 8.

Reference Type DERIVED
PMID: 30742809 (View on PubMed)

Guenther A, Krauss E, Tello S, Wagner J, Paul B, Kuhn S, Maurer O, Heinemann S, Costabel U, Barbero MAN, Muller 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.

Reference Type DERIVED
PMID: 30055613 (View on PubMed)

Related Links

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http://www.pulmonary-fibrosis.net/

European IPF Registry website

Other Identifiers

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EurIPFreg_150609

Identifier Type: -

Identifier Source: org_study_id

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