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

Andreas Guenther

Status: Active, not recruiting

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

Treatments: Other: patient registry (observation and biomaterial sampling)

Study type: Observational

Funder types: Other

Identifiers

NCT02951416

EurIPFreg_150609

Details and patient eligibility

About

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/).

Full 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.

Enrollment: 2,000 estimated patients

Sex: All

Ages: 18+ years old

Volunteers: Accepts Healthy Volunteers

Inclusion criteria

• Informed consent signed

Exclusion criteria

• No informed consent signed

Trial design

2,000 participants in 15 patient groups

Idiopathic Pulmonary Fibrosis (IPF)

Description:

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).

Treatment:

Other: patient registry (observation and biomaterial sampling)

Non-specific interstitial pneumonia

Description:

Non-specific interstitial pneumonia (NSIP) based on the histopathological demonstration of an NSIP pattern. Patient registry (observation and biomaterial sampling).

Treatment:

Other: patient registry (observation and biomaterial sampling)

Cryptogenic organising pneumonia (COP)

Description:

COP characterised histologically by an organising pneumonia with intraluminal organising fibrosis in the alveolar ducts and alveolar spaces. Patient registry (observation and biomaterial sampling).

Treatment:

Other: patient registry (observation and biomaterial sampling)

Acute interstitial pneumonia (AIP)

Description:

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).

Treatment:

Other: patient registry (observation and biomaterial sampling)

Lymphoid interstitial pneumonia (LIP)

Description:

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).

Treatment:

Other: patient registry (observation and biomaterial sampling)

respiratory bronchiolitis-ILD (RB-ILD)

Description:

Histological pattern of RB-ILD or typical clinical and radiological findings. Patient registry (observation and biomaterial sampling).

Treatment:

Other: patient registry (observation and biomaterial sampling)

Desquamative Interstitial Pneumonia

Description:

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).

Treatment:

Other: patient registry (observation and biomaterial sampling)

Hypersensitivity Pneumonitis

Description:

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).

Treatment:

Other: patient registry (observation and biomaterial sampling)

Sarcoidosis

Description:

Histological pattern with sarcoid granulomas or typical clinical and radiological findings with a lymphocytosis in BAL. Patient registry (observation and biomaterial sampling).

Treatment:

Other: patient registry (observation and biomaterial sampling)

Lung Cancer

Description:

Histological confirmation of Lung Cancer. Patients will be included as control group. Patient registry (observation and biomaterial sampling).

Treatment:

Other: patient registry (observation and biomaterial sampling)

Chronic Obstructive Pulmonary Disease

Description:

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

Treatment:

Other: patient registry (observation and biomaterial sampling)

Pulmonary Hypertension

Description:

Pulmonary Hypertension (PH) diagnosed through right heart catheterisation. Patients will be included as control group. Patient registry (observation and biomaterial sampling).

Treatment:

Other: patient registry (observation and biomaterial sampling)

Sleep Apnea

Description:

Sleep Apnea diagnosed by polysomnography. Patients will be included as control group. Patient registry (observation and biomaterial sampling).

Treatment:

Other: patient registry (observation and biomaterial sampling)

Asthma

Description:

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).

Treatment:

Other: patient registry (observation and biomaterial sampling)

Control/Health Individuals

Description:

Healthy volunteers not suffering from any lung disease as control group. Patient registry (observation and biomaterial sampling).

Treatment:

Other: patient registry (observation and biomaterial sampling)