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

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ClinicalTrials.gov Identifier: NCT02951416
Recruitment Status : Recruiting
First Posted : November 1, 2016
Last Update Posted : November 20, 2017
Sponsor:
Information provided by (Responsible Party):
Andreas Guenther, University of Giessen

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 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


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




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


Information from the National Library of Medicine

Choosing to participate in a study is an important personal decision. Talk with your doctor and family members or friends about deciding to join a study. To learn more about this study, you or your doctor may contact the study research staff using the contacts provided below. For general information, Learn About Clinical Studies.


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

Information from the National Library of Medicine

To learn more about this study, you or your doctor may contact the study research staff using the contact information provided by the sponsor.

Please refer to this study by its ClinicalTrials.gov identifier (NCT number): NCT02951416


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
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

Additional Information:
Publications:
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    Key Record Dates
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:
Pneumonia
Lung Diseases
Pulmonary Fibrosis
Idiopathic Pulmonary Fibrosis
Idiopathic Interstitial Pneumonias
Lung Diseases, Interstitial
Respiratory Tract Diseases
Respiratory Tract Infections