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

NCT02951416 | Active Not Recruiting

• 1 other identifier: EurIPFreg_150609
• Study Type: observational
• Target: 2,000
• Locations: 5 countries
• Sites: 7

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/).

Conditions

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

Outcome Measures

Primary Outcomes (1)

• clinical course of patients with Interstitial Lung Diseases (ILD)

  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 Outcomes (4)

• Comorbidities of patients with ILD

  5 years

• Infections in lung function of patients with ILD

  5 years

• Quality of life of patients with ILD

  5 years

• Health care utilization of patients with ILD

  5 years

Study Arms (15)

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)

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)

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)

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)

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)

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)

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)

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)

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)

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)

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)

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)

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)

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)

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)

Interventions

patient registry (observation and biomaterial sampling) OTHER

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

Acute interstitial pneumonia (AIP); Asthma; Chronic Obstructive Pulmonary Disease; Control/Health Individuals; Cryptogenic organising pneumonia (COP); Desquamative Interstitial Pneumonia; Hypersensitivity Pneumonitis; Idiopathic Pulmonary Fibrosis (IPF); Lung Cancer; Lymphoid interstitial pneumonia (LIP); Non-specific interstitial pneumonia; Pulmonary Hypertension; Sarcoidosis; Sleep Apnea; respiratory bronchiolitis-ILD (RB-ILD)

Eligibility Criteria

• Age: 18 Years+
• Sex: all
• Healthy Volunteers: Yes
• Age Groups: Adult (18-64), Older Adult (65+)
• Sampling Method: Probability Sample

Study Population

Patients with Interstitial Lung Diseases

You may qualify if:

• Informed consent signed

You may not qualify if:

• No informed consent signed

Sponsors & Collaborators

• Andreas Guenther: lead

Study Sites (7)

Medizinische Universität Wien

Vienna, Austria

Location

Centre Hospitalier Universitaire Dijon

Dijon, France

Location

Hopital Bichat Paris

Paris, France

Location

Andreas Guenther

Giessen, 35392, Germany

Location

Lungenfachklinik Waldhof Elgershausen

Greifenstein, Germany

Location

Università degli Studi di Catania

Catania, Italy

Location

Royal Brompton Hospital

London, United Kingdom

Location

Related Publications (9)

• 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.

  PMID: 21454892 BACKGROUND

• 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.

  PMID: 25551350 BACKGROUND

• 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.

  PMID: 39879227 DERIVED

• 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.

  PMID: 39558302 DERIVED

• 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.

  PMID: 32325432 DERIVED

• 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.

  PMID: 31319833 DERIVED

• 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.

  PMID: 30823880 DERIVED

• 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.

  PMID: 30742809 DERIVED

• 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.

  PMID: 30055613 DERIVED

Related Links

• European IPF Registry website

Biospecimen

Retention: SAMPLES WITH DNA

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

MeSH Terms

Conditions

Idiopathic Pulmonary Fibrosis; Idiopathic Interstitial Pneumonias; Lung Diseases, Interstitial

Interventions

Observation

Condition Hierarchy (Ancestors)

Pulmonary Fibrosis; Lung Diseases; Respiratory Tract Diseases

Intervention Hierarchy (Ancestors)

Methods; Investigative Techniques

Study Design

• Study Type: observational
• Observational Model: COHORT
• Time Perspective: CROSS SECTIONAL
• Target Duration: 60 Months
• Sponsor Type: OTHER
• Responsible Party: SPONSOR INVESTIGATOR
• PI Title: Professor of Internal Medicine

Study Record Dates

• First Submitted: June 9, 2015
• First Posted: November 1, 2016
• Study Start: September 1, 2009
• Primary Completion: January 1, 2025
• Study Completion (Estimated): January 1, 2040
• Last Updated: February 28, 2024

Record last verified: 2024-02

Locations

FR (2); AU (1); DE (2); IT (1); GB (1)