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

Study Overview
=================
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/).

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.

Official Title
-----------------
The European IPF Registry - an Internet-based, Pan-European Registry Linked to the European IPF Biobank (eurIPFbank)

Conditions
-----------------
Idiopathic Pulmonary Fibrosis, Idiopathic Interstitial Pneumonia, Interstitial Lung Diseases, Diffuse Parenchymal Lung Diseases

Intervention / Treatment
-----------------
* Other: patient registry (observation and biomaterial sampling)


Participation Criteria
=================
Eligibility Criteria
-----------------
Inclusion Criteria: Informed consent signed Exclusion Criteria: No informed consent signed

Ages Eligible for Study
-----------------
Minimum Age: 18 Years

Sexes Eligible for Study
-----------------
All

Accepts Healthy Volunteers
-----------------
Accepts Healthy Volunteers

Study Plan
=================
How is the study designed?
-----------------

Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Idiopathic Pulmonary Fibrosis (IPF)<br>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)<br>* data are collected with patient questionnaires, additionally clinical data are collected at every routine visit and biomaterial is collected<br>|
| Non-specific interstitial pneumonia<br>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)<br>* data are collected with patient questionnaires, additionally clinical data are collected at every routine visit and biomaterial is collected<br>|
| Cryptogenic organising pneumonia (COP)<br>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)<br>* data are collected with patient questionnaires, additionally clinical data are collected at every routine visit and biomaterial is collected<br>|
| Acute interstitial pneumonia (AIP)<br>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)<br>* data are collected with patient questionnaires, additionally clinical data are collected at every routine visit and biomaterial is collected<br>|
| Lymphoid interstitial pneumonia (LIP)<br>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)<br>* data are collected with patient questionnaires, additionally clinical data are collected at every routine visit and biomaterial is collected<br>|
| respiratory bronchiolitis-ILD (RB-ILD)<br>Histological pattern of RB-ILD or typical clinical and radiological findings. Patient registry (observation and biomaterial sampling). | Other: patient registry (observation and biomaterial sampling)<br>* data are collected with patient questionnaires, additionally clinical data are collected at every routine visit and biomaterial is collected<br>|
| Desquamative Interstitial Pneumonia<br>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)<br>* data are collected with patient questionnaires, additionally clinical data are collected at every routine visit and biomaterial is collected<br>|
| Hypersensitivity Pneumonitis<br>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)<br>* data are collected with patient questionnaires, additionally clinical data are collected at every routine visit and biomaterial is collected<br>|
| Sarcoidosis<br>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)<br>* data are collected with patient questionnaires, additionally clinical data are collected at every routine visit and biomaterial is collected<br>|
| Lung Cancer<br>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)<br>* data are collected with patient questionnaires, additionally clinical data are collected at every routine visit and biomaterial is collected<br>|
| Chronic Obstructive Pulmonary Disease<br>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)<br>* data are collected with patient questionnaires, additionally clinical data are collected at every routine visit and biomaterial is collected<br>|
| Pulmonary Hypertension<br>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)<br>* data are collected with patient questionnaires, additionally clinical data are collected at every routine visit and biomaterial is collected<br>|
| Sleep Apnea<br>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)<br>* data are collected with patient questionnaires, additionally clinical data are collected at every routine visit and biomaterial is collected<br>|
| Asthma<br>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)<br>* data are collected with patient questionnaires, additionally clinical data are collected at every routine visit and biomaterial is collected<br>|
| Control/Health Individuals<br>Healthy volunteers not suffering from any lung disease as control group. Patient registry (observation and biomaterial sampling). | Other: patient registry (observation and biomaterial sampling)<br>* data are collected with patient questionnaires, additionally clinical data are collected at every routine visit and biomaterial is collected<br>|

What is the study measuring?
-----------------
Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| 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 Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Comorbidities of patients with ILD | reported in patients and physicians questionnaires | 5 years | 
| Infections in lung function of patients with ILD | reported in patients and physicians questionnaires | 5 years | 
| Quality of life of patients with ILD | reported in patients and physicians questionnaires, EQ5D (European quality of life 5-dimensions) questionnaire | 5 years | 
| Health care utilization of patients with ILD | reported in patients questionnaires | 5 years |