Taiwan Interstitial Lung Disease Multi-center Investigation and Registry

A Prospective Multi-center Registry for Fibrotic Lung Disease, Focusing on Clinical Phenotype, Physical Parameters, Image Analysis, and Precision Medicine in Taiwan

The Taiwan Interstitial Lung Disease (ILD) Multi-center Investigation and Registry aims to evaluate the long-term outcomes of patients with fibrotic interstitial lung disease. This prospective observational registry will collect comprehensive clinical data from multiple centers, including epidemiological information, comorbidities, questionnaire results, routine blood tests, biochemical tests, pulmonary function tests, echocardiograms, and cardiopulmonary exercise tests (CPET), all following a standardized protocol.

Key components of the registry include annual HRCT scans, annual CPETs, biobank blood samples, and biannual echocardiograms and pulmonary function tests.

The main questions the registry aims to answer are:

1. Differences in all-cause mortality among ILD patients of different etiologies.
2. Differences in the annual risk of acute exacerbation among ILD patients of different etiologies.
3. Effectiveness of current anti-fibrotic drugs in treating IPF and ILD of different etiologies.
4. Predictive ability of HRCT imaging features for mortality risk in ILD patients.
5. Impact of comorbidities on the mortality risk of ILD patients.
6. Predictive ability of biomarkers for disease progression and mortality.

The goal of this prospective observational registry is to evaluate the long-term outcomes of patients diagnosed with fibrotic interstitial lung disease in Taiwan.

The investigators will conduct a prospective registry and collect clinical data of fibrotic lung disease patients from multiple centers in Taiwan. The data collected will include basic epidemiological information, comorbidities, questionnaire results, routine blood tests, biochemical tests, pulmonary function tests, echocardiograms, and cardiopulmonary exercise tests (CPET). Each hospital will follow the same protocol for data collection, establishing a real-world Taiwan Fibrotic Lung Disease Registry Database.

The details of this registry plan include:

1. Annual high-resolution computed tomography (HRCT) scans
2. Annual cardiopulmonary exercise tests (CPET)
3. Peripheral blood sampling for inclusion in a biobank
4. Additionally, echocardiograms and pulmonary function tests will be conducted every six months.

The main questions it aims to answer are:

1. Is there a difference in all-cause mortality among ILD patients of different etiologies?
2. Is there a difference in the annual risk of acute exacerbation among ILD patients of different etiologies?
3. Evaluate the real-world data on the effectiveness of current anti-fibrotic drugs in treating idiopathic pulmonary fibrosis (IPF) and ILD of different etiologies.
4. Investigate the predictive ability of high-resolution computed tomography (HRCT) imaging features for mortality risk in ILD patients.
5. Explore the impact of comorbidities on the mortality risk of ILD patients.
6. Assess the predictive ability of biomarkers for disease progression and mortality.

Inclusion Criteria:

1. Aged over 18 years old.
2. Diagnosed as ILD by a pulmonologist, rheumatologist or radiologist
3. Various casue of ILD, including Idiopathic pulmonary fibrosis (IPF), Connective tissue disease-associated interstitial lung disease (CTD-ILD), Unclassifed ILD, drug-induced ILD, lymphangioleiomyomatosis (LAM), and sarcoidosis-associated ILD.

Exclusion Criteria:

1. Under 18 years of age.
2. Failure to express informed consent in person.