{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,5]],"date-time":"2026-10-05T22:50:29Z","timestamp":1791240629772,"version":"4.1.0"},"reference-count":60,"publisher":"The Korean Academy of Tuberculosis and Respiratory Diseases","issue":"4","license":[{"start":{"date-parts":[[2026,6,24]],"date-time":"2026-06-24T00:00:00Z","timestamp":1782259200000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"name":"Korean Academy of Tuberculosis and Respiratory Diseases","award":["KATRD-S-2022-1"],"award-info":[{"award-number":["KATRD-S-2022-1"]}]},{"DOI":"10.13039\/501100003725","name":"National Research Foundation of Korea","doi-asserted-by":"publisher","award":["2022M3A9E4082647"],"award-info":[{"award-number":["2022M3A9E4082647"]}],"id":[{"id":"10.13039\/501100003725","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Ministry of Science and ICT","id":[{"id":"https:\/\/ror.org\/01wpjm123","id-type":"ROR","asserted-by":"crossref"}]},{"DOI":"10.13039\/501100003653","name":"Korea National Institute of Health","doi-asserted-by":"publisher","award":["2024ER090500"],"award-info":[{"award-number":["2024ER090500"]}],"id":[{"id":"10.13039\/501100003653","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Korea Environment Industry & Technology Institute"},{"DOI":"10.13039\/501100003562","name":"Ministry of Environment","doi-asserted-by":"publisher","award":["RS-2022-KE002197"],"award-info":[{"award-number":["RS-2022-KE002197"]}],"id":[{"id":"10.13039\/501100003562","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["e-trd.org"],"crossmark-restriction":true},"short-container-title":["Tuberc Respir Dis"],"published-print":{"date-parts":[[2026,10,1]]},"abstract":"<jats:p>Background: Rare interstitial lung diseases (ILDs) are a heterogeneous group of diffuse parenchymal lung disorders with low prevalence and diverse etiologies that pose diagnostic and therapeutic challenges. We aimed to establish a nationwide, multicenter registry of rare ILDs in South Korea to characterize their clinical features and outcomes.Methods: We conducted a multicenter, observational cohort study involving 304 patients with rare ILDs including Birt\u2013Hogg\u2013Dub\u00e9 syndrome (BHD), lymphangioleiomyomatosis (LAM), pulmonary alveolar proteinosis (PAP), pulmonary Langerhans cell histiocytosis (PLCH), pleuroparenchymal fibroelastosis (PPFE), and hypersensitivity pneumonitis (HP) enrolled across 20 centers in Korea between September 2023 and December 2024. Data on clinical characteristics, outcomes, and treatment were collected.Results: LAM (28.3%) and PAP (26.6%) were the most prevalent, followed by BHD (18.1%), PLCH (13.2%), HP (7.6%), and PPFE (6.3%). Dyspnea was common in HP and PPFE, whereas BHD and LAM were often asymptomatic. Pneumothorax was frequent in BHD (44.6%) and LAM (11.6%) at diagnosis. PPFE showed the most impaired lung function and the highest mortality (26.3%), during a median follow-up of 69.5 months, followed by PAP (6.2%) and BHD (1.8%). During follow-up, cancer was reported in 25.5% of patients with BHD. Treatment strategies varied by disease: corticosteroids were commonly used in HP (86.9%), PPFE (42.1%), and PLCH (32.5%); sirolimus in LAM (53.5%); pirfenidone in PPFE (42.1%); and whole-lung lavage in PAP (23.5%).Conclusion: This is the first nationwide rare ILD registry in Korea, demonstrating heterogeneity in clinical features and outcomes and emphasizing the need for disease-specific diagnostic and therapeutic strategies.<\/jats:p>","DOI":"10.4046\/trd.2026.0004","type":"journal-article","created":{"date-parts":[[2026,6,24]],"date-time":"2026-06-24T06:56:31Z","timestamp":1782284191000},"page":"529-541","update-policy":"https:\/\/doi.org\/10.4046\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["A Multicenter Registry for Rare Interstitial Lung Diseases in Korea: Baseline Characteristics and Clinical Outcomes"],"prefix":"10.4046","volume":"89","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0756-3810","authenticated-orcid":true,"given":"Ju Hyun","family":"Oh","sequence":"first","affiliation":[{"name":"Department of Pulmonology and Critical Care Medicine, Ajou University School of Medicine, Suwon, Republic of Korea"},{"name":"Department of Pulmonary and Critical Care Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Republic of Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0932-2826","authenticated-orcid":true,"given":"Jae Ha","family":"Lee","sequence":"additional","affiliation":[{"name":"Division of Pulmonology, Department of Internal Medicine, Inje University Haeundae Paik Hospital, Inje University College of Medicine, Busan, Republic of Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sung Woo","family":"Park","sequence":"additional","affiliation":[{"name":"Division of Allergy and Respiratory Medicine, Department of Internal Medicine, Soonchunhyang University Bucheon Hospital, Bucheon, Republic of Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yong Suk","family":"Jo","sequence":"additional","affiliation":[{"name":"Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Seoul St. Mary\u2019s Hospital, College of Medicine, The Catholic University of Korea, Seoul, Republic of Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jong Sun","family":"Park","sequence":"additional","affiliation":[{"name":"Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Seoul National University Bundang Hospital, Seongnam, Republic of Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hongseok","family":"Yoo","sequence":"additional","affiliation":[{"name":"Division of Pulmonary and Critical Care Medicine, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Republic of Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kyung Hoon","family":"Kim","sequence":"additional","affiliation":[{"name":"Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Incheon St. Mary\u2019s Hospital, College of Medicine, The Catholic University of Korea, Seoul, Republic of Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yangin","family":"Jegal","sequence":"additional","affiliation":[{"name":"Division of Pulmonary and Critical Care Medicine, Ulsan University Hospital, University of Ulsan College of Medicine, Ulsan, Republic of Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joo Hun","family":"Park","sequence":"additional","affiliation":[{"name":"Department of Pulmonology and Critical Care Medicine, Ajou University School of Medicine, Suwon, Republic of Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jinkyeong","family":"Park","sequence":"additional","affiliation":[{"name":"Department of Pulmonary, Allergy and Critical Care Medicine, Kyung Hee University Hospital at Gangdong, Seoul, Republic of Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Taehun","family":"Kim","sequence":"additional","affiliation":[{"name":"Division of Pulmonology, Department of Internal Medicine, Keimyung University Dongsan Hospital, Keimyung University School of Medicine, Daegu, Republic of Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bo-Guen","family":"Kim","sequence":"additional","affiliation":[{"name":"Division of Pulmonary Medicine, Department of Internal Medicine, Kangbuk Samsung Hospital, Sungkyunkwan University School of Medicine, Seoul, Republic of Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hyung Koo","family":"Kang","sequence":"additional","affiliation":[{"name":"Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Inje University Ilsan Paik Hospital, Inje University College of Medicine, Goyang, Republic of Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hyun-Kyung","family":"Lee","sequence":"additional","affiliation":[{"name":"Division of Pulmonary, Allergy, and Critical Care Medicine, Department of Internal Medicine, Inje University Busan Paik Hospital, Busan, Republic of Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ji Young","family":"Kang","sequence":"additional","affiliation":[{"name":"Division of Pulmonary and Critical Care Medicine, Cheju Halla General Hospital, Jeju, Republic of Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hee-Young","family":"Yoon","sequence":"additional","affiliation":[{"name":"Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Soonchunhyang University Seoul Hospital, Seoul, Republic of Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hye Ju","family":"Yeo","sequence":"additional","affiliation":[{"name":"Transplant Research Center, Research Institute for Convergence of Biomedical Science and Technology, Pusan National University Yangsan Hospital and Division of Allergy, Pulmonary and Critical Care Medicine, Department of Internal Medicine, Pusan National University School of Medicine, Yangsan, Republic of Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dong Won","family":"Park","sequence":"additional","affiliation":[{"name":"Division of Pulmonary Medicine and Allergy, Department of Internal Medicine, Hanyang University Medical Center, Hanyang University College of Medicine, Seoul, Republic of Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ala","family":"Woo","sequence":"additional","affiliation":[{"name":"Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Severance Hospital, Yonsei University College of Medicine, Seoul, Republic of Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jung-Wan","family":"Yoo","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Gyeongsang National University Hospital, Gyeongsang National University College of Medicine, Jinju, Republic of Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5121-3522","authenticated-orcid":true,"given":"Jin Woo","family":"Song","sequence":"additional","affiliation":[{"name":"Department of Pulmonary and Critical Care Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Republic of Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"name":"on behalf of the Rare ILD Registry 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