Clinical trial NCT06476470: Taiwan Interstitial Lung Disease Multi-center Investigation and Registry
Official title: A Prospective Multi-center Registry for Fibrotic Lung Disease, Focusing on Clinical Phenotype, Physical Parameters, Image Analysis, and Precision Medicine in Taiwan
Study type: OBSERVATIONAL
Overall status: RECRUITING
Conditions: Interstitial Lung Disease
Comorbidities and Coexisting Conditions
Pulmonary Arterial Hypertension
Treatment Adherence
Function Impaired Respiratory
Lead sponsor: Taichung Veterans General Hospital
Sponsor class: OTHER
Collaborators: name: Tri-Service General Hospital
name: E-DA Hospital
name: Chang Gung Memorial Hospital
First posted: 2024-06-26
Enrollment: 10000
Sex: ALL
Minimum age (years): 18
Standard ages: ADULT
OLDER_ADULT
Accepts healthy volunteers: no
Observational model: COHORT
Time perspective: PROSPECTIVE
Sampling method: NON_PROBABILITY_SAMPLE
Study population: Enroll patients with various causes of fibrotic lung diseases, including idiopathic pulmonary fibrosis (IPF), connective tissue disease-associated interstitial lung disease (CTD-ILD), unclassified ILD, pneumoconiosis, drug-induced ILD, lymphangioleiomyomatosis (LAM), and sarcoidosis-associated ILD.

All included ILD cases must be diagnosed through a multidisciplinary discussion at each hospital. The specialists involved should include at least a pulmonologist, rheumatologist, and radiologist. If a patient has a pathology diagnosis, the pathologist should also provide their recommendations and expert opinions.

Brief summary: 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.

Detailed description: 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.

Eligibility criteria: 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.

Primary outcomes:
measure: All-cause mortality
timeFrame: 10 years
measure: Annual rate of acute exacerbation
timeFrame: through study completion, an average of 1 year
Secondary outcomes:
measure: Annual rate of progressive pulmonary fibrosis
timeFrame: From date of enrolled until the date of first documented progression or date of death from any cause, whichever came first, assessed up to 120 months
Other outcomes:
Locations:
city: Taichung
country: Taiwan
facility: Taichung Veterans General Hospital
Study officials:
affiliation: Taichung Veterans General Hospital
name: Pin-Kuei Fu, MD., Ph.D
role: PRINCIPAL_INVESTIGATOR
References:
citation: Chen YW, Lai CH, Liao YW, Liu MC, Wu YC, Hsu CY, Yu YH, Fu PK. A composite score based on cardiovascular parameters can predict the mortality risk of patients with newly diagnosed interstitial lung disease: A prospective observational study. J Cardiol. 2024 Oct;84(4):287-293. doi: 10.1016/j.jjcc.2024.03.012. Epub 2024 Apr 4.
citation: Liao YW, Liu MC, Wu YC, Hsu CY, Huang WN, Chen YH, Fu PK. Factors influencing long-term outcomes in fibrotic interstitial lung disease (F-ILD) diagnosed through multidisciplinary discussion (MDD): a prospective cohort study. Eur J Med Res. 2024 Jan 30;29(1):91. doi: 10.1186/s40001-024-01673-2.
citation: Liao YW, Chen YM, Liu MC, Wu YC, Hsu CY, Fu PK, Huang WN, Chen YH. Multidisciplinary-derived clinical score for accurate prediction of long-term mortality in fibrotic lung disease patients. Eur J Med Res. 2024 Jan 20;29(1):69. doi: 10.1186/s40001-024-01644-7.