Japanese Idiopathic Interstitial Pneumonias Registry

Study Overview
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Brief Summary
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An objective of JIPS Registry is to examine disease behavior of idiopathic interstitial pneumonias (IIPs), considering classification, background, and diagnostic methods based on American Thoracic Society (ATS)/ European Respiratory Society(ERS) /Japanese Respiratory Society (JRS)/ Latin American Thoracic Association (ALAT) guidelines for diagnosis and the ATS/ERS classification of 2002 and 2013.

Detailed Description
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JIPS Registry is a multi-site, non-interventional, prospective observation study of patients with newly diagnosed IIPs in Japan. At least 600 patients will be registered for 16 months at approximately 80 sites in Japan. Primary research question is to determine the natural history of each category of IIPs at registration, patient background and diagnosis methods will be considered. Furthermore, the present treatment patterns and disease behavior (CT and forced vital capacity (FVC) changes, as well as changes in interstitial pneumonia markers, etc.) will also be investigated.

Official Title
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Japanese Idiopathic Interstitial Pneumonias Registry

Conditions
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Idiopathic Interstitial Pneumonia

Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Patients aged between 20 and 84 years Patients with idiopathic pulmonary fibrosis (IPF) and other IIPs diagnosis within 6 months before registration at each facility Patients from whom written informed consent has been obtained regarding participation in this study and follow-up observation Exclusion Criteria: Patients for whom the tests (such as the lung function test) conducted in this study could not be performed Patients who underwent pulmonary resection Patients undergoing dialysis Patients with cancer treated at the time of registration or those planning to receive treatment in the future

Ages Eligible for Study
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Minimum Age: 20 Years
Maximum Age: 84 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Data on current practice patterns for diagnosis of IIPs | Describe the frequency of surgical lung biopsy, bronchoalveolar lavage. | 3-4 years | 
| Prevalence of each categorized IIPs | Investigate the prevalence of each categorized IIPs by using a recent guideline | 3-4 years | 
| Natural history of each categorized IIPs | Describe the natural history of each categorized IIPs, focusing on CT findings, FVC changes, medications, and causes of death. | 3-4 years | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Progression-free survival by category of IIPs |  | 3-6 years | 
| Hospital admission for acute exacerbations of IIPs | To evaluate incidences, therapy and prognosis of acute respiratory deterioration in patients with IIPs | 3-6 years | 
| Mean change of patient-reported outcome (Saint George Respiratory Questionnaire, COPD assessment test, and Dyspnoea-12) every12 months from baseline |  | 3-4 years | 
| Quantitative evaluation of CT findings |  | 3-6 years | 
| Validation of second multidisciplinary discussion | Multidisciplinary discussion (MDD) held by independent central reviewers will be conducted twice at the time of registration and final observation using questionnaire. Concordance rate for diagnosis and disease behavior in each case between the time of registration and final observation will be evaluated. | 3-4 years | 
| Validation of new guideline | If there are changes in IIPs diagnostic guidelines or severity classification during this study, these changes will be verified. | 3-6 years | 
| Diagnosis of IIPs in patients with IIPs at registration | Describe the frequency of IIPs diagnosis using data at the time of registration. | At registration and 1 year after registration | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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IIPs