{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T10:58:50Z","timestamp":1790852330078,"version":"4.1.0"},"reference-count":13,"publisher":"MDPI AG","issue":"19","license":[{"start":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T00:00:00Z","timestamp":1790812800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J. Clin. Med.","JCM"],"abstract":"<jats:p>Background\/Objectives: Anti-melanoma differentiation-associated gene 5 (anti-MDA5) antibody is associated with dermatomyositis\/clinically amyopathic dermatomyositis and a high risk of rapidly progressive interstitial lung disease (RP-ILD). Methods: We retrospectively analyzed 34 consecutive anti-MDA5-positive patients evaluated at a tertiary Advanced Lung Disease and Lung Transplant center between 1 January 2018 and 31 December 2024. Results: Of 1720 interstitial lung disease (ILD) evaluations, 34 (2.0%) were anti-MDA5 positive (median age 51 years; 56% female). ILD was present in 85%; RP-ILD developed in 13 patients (38%). Overall mortality was 32%, with 11 of 13 deaths occurring in the RP-ILD subgroup (85% mortality). Six patients were listed for lung transplantation and three underwent transplantation; all three remained alive at last follow-up (24\u201349 months). Right-heart catheterization performed in 11 patients demonstrated pulmonary hypertension (PH) in the majority (64%). Inpatient presentation and elevated ferritin were associated with RP-ILD; elevated CRP and RP-ILD predicted mortality. Conclusions: We conclude that anti-MDA-5-associated ILD carries high mortality when RP-ILD develops. Early recognition, aggressive immunosuppression, systematic consideration of PH, and timely referral for lung transplantation can improve outcomes in selected patients. Routine screening for anti-MDA-5 antibodies should be considered in younger patients with new or progressive ILD.<\/jats:p>","DOI":"10.3390\/jcm15197623","type":"journal-article","created":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T10:25:44Z","timestamp":1790850344000},"page":"7623","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Clinical Characteristics and Outcomes of Anti-MDA5 Antibody-Associated Interstitial Lung Disease: A Single-Center Retrospective Cohort Study from an Integrated Advanced Lung Disease and Lung Transplant Program in the US"],"prefix":"10.3390","volume":"15","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3753-4378","authenticated-orcid":false,"given":"Shambhu","family":"Aryal","sequence":"first","affiliation":[{"name":"Advanced Lung Disease and Transplant Program, Inova Schar Heart and Vascular Institute, Inova Health System, 3300 Gallows Rd, Falls Church, VA 22042, USA"}],"role":[{"vocabulary":"crossref","role":"author"},{"role":"conceptualization","vocabulary":"credit"},{"role":"methodology","vocabulary":"credit"},{"role":"validation","vocabulary":"credit"},{"role":"formal-analysis","vocabulary":"credit"},{"role":"investigation","vocabulary":"credit"},{"role":"writing-review-editing","vocabulary":"credit"},{"role":"supervision","vocabulary":"credit"}]},{"given":"Shourjo","family":"Chakravorty","sequence":"additional","affiliation":[{"name":"Division of Pulmonary and Critical Care Medicine, Thomas Jefferson Hospital, 111 S 11th S, Philadelphia, PA 19107, USA"}],"role":[{"vocabulary":"crossref","role":"author"},{"role":"data-curation","vocabulary":"credit"},{"role":"writing-review-editing","vocabulary":"credit"}]},{"given":"Ho Cheol","family":"Kim","sequence":"additional","affiliation":[{"name":"Department of Pulmonary & Critical Care Medicine, Asan Medical Center, University of Ulsan College of Medicine, 88, Olympic-ro 43-gil, Songpa-gu, Seoul 05505, Republic of Korea"}],"role":[{"vocabulary":"crossref","role":"author"},{"role":"data-curation","vocabulary":"credit"},{"role":"writing-review-editing","vocabulary":"credit"}]},{"given":"Ashima","family":"Malik","sequence":"additional","affiliation":[{"name":"Division of Rheumatology, Inova Fairfax Hospital, 3300 Gallows Rd, Falls Church, VA 22042, USA"}],"role":[{"vocabulary":"crossref","role":"author"},{"role":"writing-review-editing","vocabulary":"credit"},{"role":"conceptualization","vocabulary":"credit"}]},{"given":"Pragya","family":"Singh","sequence":"additional","affiliation":[{"name":"Division of Rheumatology, Inova Fairfax Hospital, 3300 Gallows Rd, Falls Church, VA 22042, USA"}],"role":[{"vocabulary":"crossref","role":"author"},{"role":"writing-review-editing","vocabulary":"credit"},{"role":"conceptualization","vocabulary":"credit"}]},{"given":"Ramona","family":"Raya","sequence":"additional","affiliation":[{"name":"Division of Rheumatology, Inova Fairfax Hospital, 3300 Gallows Rd, Falls Church, VA 22042, USA"}],"role":[{"vocabulary":"crossref","role":"author"},{"role":"conceptualization","vocabulary":"credit"},{"role":"writing-review-editing","vocabulary":"credit"}]},{"given":"Oksana A.","family":"Shlobin","sequence":"additional","affiliation":[{"name":"Advanced Lung Disease and Transplant Program, Inova Schar Heart and Vascular Institute, Inova Health System, 3300 Gallows Rd, Falls Church, VA 22042, USA"}],"role":[{"vocabulary":"crossref","role":"author"},{"role":"writing-review-editing","vocabulary":"credit"}]},{"given":"Christopher S.","family":"King","sequence":"additional","affiliation":[{"name":"Advanced Lung Disease and Transplant Program, Inova Schar Heart and Vascular Institute, Inova Health System, 3300 Gallows Rd, Falls Church, VA 22042, USA"}],"role":[{"vocabulary":"crossref","role":"author"},{"role":"writing-review-editing","vocabulary":"credit"}]},{"given":"Steven D.","family":"Nathan","sequence":"additional","affiliation":[{"name":"Advanced Lung Disease and Transplant Program, Inova Schar Heart and Vascular Institute, Inova Health System, 3300 Gallows Rd, Falls Church, VA 22042, USA"}],"role":[{"vocabulary":"crossref","role":"author"},{"role":"writing-review-editing","vocabulary":"credit"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9486-1674","authenticated-orcid":false,"given":"Zein","family":"Kattih","sequence":"additional","affiliation":[{"name":"Advanced Lung Disease and Transplant Program, Inova Schar Heart and Vascular Institute, Inova Health System, 3300 Gallows Rd, Falls Church, VA 22042, 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