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
4 affiliations
- 1Advanced Lung Disease and Transplant Program, Inova Schar Heart and Vascular Institute, Inova Health System, 3300 Gallows Rd, Falls Church, VA 22042, USA
- 2Division of Pulmonary and Critical Care Medicine, Thomas Jefferson Hospital, 111 S 11th S, Philadelphia, PA 19107, USA
- 3Department 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
- 4Division of Rheumatology, Inova Fairfax Hospital, 3300 Gallows Rd, Falls Church, VA 22042, USA
Abstract
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 (24–49 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.
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BibTeX
@article{aryal2026clinical,
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}},
author = {Shambhu Aryal and Shourjo Chakravorty and Ho Cheol Kim and Ashima Malik and Pragya Singh and Ramona Raya and Oksana A. Shlobin and Christopher S. King and Steven D. Nathan and Zein Kattih},
journal = {Journal of clinical medicine},
year = {2026},
month = oct,
doi = {10.3390/jcm15197623},
url = {https://europepmc.org/article/PMC/PMC13666125},
note = {CC BY},
}
RIS
TY - JOUR
TI - 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
AU - Shambhu Aryal
AU - Shourjo Chakravorty
AU - Ho Cheol Kim
AU - Ashima Malik
AU - Pragya Singh
AU - Ramona Raya
AU - Oksana A. Shlobin
AU - Christopher S. King
AU - Steven D. Nathan
AU - Zein Kattih
PY - 2026
DA - 2026/10/01
JO - Journal of clinical medicine
DO - 10.3390/jcm15197623
UR - https://europepmc.org/article/PMC/PMC13666125
AB - 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 (24–49 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.
SN - 2077-0383
ER -