Comorbidities and Coexisting Conditions, Function Impaired Respiratory, Interstitial Lung Disease, Pulmonary Arterial Hypertension, Treatment Adherence
Conditions
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.
Interventions
None listed
Sponsors
Tri-Service General Hospital
E-DA Hospital
Chang Gung Memorial Hospital
Taichung Veterans General Hospital
Study design
Observational model
COHORT
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
18 Years to No maximum
Healthy volunteers
No
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.
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| All-cause mortality | 10 years | All-cause mortality of the enrolled patients from enrollment to the death event |
| Annual rate of acute exacerbation | through study completion, an average of 1 year | The event of visiting the emergency room or being hospitalized will be recorded |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Annual rate of progressive pulmonary fibrosis | 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 | By the definition according to ATS 2022 guideline, including symptoms worsening, Lung function declined (FVC decreased more than 5 % or DLCO decreased more than 10% within one year) and the image pattern showed progression on HRCT scan |
Countries
Taiwan
Contacts
Primary ContactPin-Kuei Fu, MD., Ph.D
Outcome results
None listed