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.
Aged over 18 years old.
Diagnosed as ILD by a pulmonologist, rheumatologist or radiologist
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.
Under 18 years of age.
Failure to express informed consent in person.
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.
Cohort
Prospective
Not listed
All-cause mortality
All-cause mortality of the enrolled patients from enrollment to the death event
Annual rate of acute exacerbation
The event of visiting the emergency room or being hospitalized will be recorded
Annual rate of progressive pulmonary fibrosis
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
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Taichung Veterans General Hospital
Lead sponsor
Tri-Service General Hospital
Collaborator
E-DA Hospital
Collaborator
Chang Gung Memorial Hospital
Collaborator
