Design and rationale of the Japanese Idiopathic Interstitial Pneumonias (JIPS) Registry

Background: Numerous studies investigated patients with IPF; however, only a few exam-ined patients with idiopathic interstitial pneumonias (IIPs).Methods: The Japanese Idiopathic Interstitial Pneumonias (JIPS) Registry, which was initi-ated in December 2016, is a multicenter prospective observational study of patients newly diagnosed with IIPs from 86 facilities treating ILDs. The plan is to enroll more than 600 new patients during the 2-year enrolment period and to follow their progress for 3 years after the last case enrolment. If additional consent is obtained, the study will continue for another 2 years. Research questions mainly focus on identifying the frequency by IIP classification, patient background, and diagnostic methods during enrolment, survival, acute exacerbation rate, changes in high-resolution CT imaging, forced vital capacity, and interstitial pneumonia markers over time. Other research questions, including those regarding disease behavior in patients with progressive fibrosing-ILD and new biomarkers associated with genetic predispositions, will be investigated.Discussion: The JIPS Registry will provide a comprehensive description of the disease pro-gression, prognosis, treatment status, new biomarkers, and validity of guidelines and central multidisciplinary decisions for IPF and similar diseases that can be differentiated from IPF among IIPs.Ethics and dissemination: Ethical approval was obtained from the institutional review board of Kanagawa Cardiovascular and Respiratory Center (KCRC-16-0005), and that of Jichi Medical University approved the biobank part (I18-005). Results will be published in peer -reviewed journals and will be presented at national and international conferences.Trial registration: ClinTrials.gov Registry (NCT03041623, first posted on February 3, 2017).(c) 2022 The Japanese Respiratory Society. Published by Elsevier B.V. All rights reserved.