Antimelanoma differentiation-associated gene 5 (anti-MDA5) antibody-positive dermatomyositis: clinical features and outcomes in a racially diverse patient cohort
Abstract Background The anti-melanoma differentiation-associated gene 5 (anti-MDA5) antibody-positive dermatomyositis is known for its association with rapidly progressive interstitial lung disease (RP-ILD) and ulcerative skin lesions, often presenting with or without muscle involvement. The aim of...
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BMC
2025-01-01
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| Series: | BMC Rheumatology |
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| Online Access: | https://doi.org/10.1186/s41927-025-00455-5 |
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| author | Sai K. Koyoda Fatema Ezzy Dawn Wahezi Anand Kumthekar Xianhong Xie Clement E. Tagoe Bibi Ayesha |
| author_facet | Sai K. Koyoda Fatema Ezzy Dawn Wahezi Anand Kumthekar Xianhong Xie Clement E. Tagoe Bibi Ayesha |
| author_sort | Sai K. Koyoda |
| collection | DOAJ |
| description | Abstract Background The anti-melanoma differentiation-associated gene 5 (anti-MDA5) antibody-positive dermatomyositis is known for its association with rapidly progressive interstitial lung disease (RP-ILD) and ulcerative skin lesions, often presenting with or without muscle involvement. The aim of this study was to identify distinct clinical and laboratory features that could be used to evaluate disease progression in an ethnically diverse cohort of anti-MDA5 dermatomyositis patients at a U.S. academic center. Methods A retrospective chart review was conducted on dermatomyositis patients hospitalized at our institution between January 2014 and June 2023. The data were analyzed via Fischer’s exact test and a t test. Results Among the 195 dermatomyositis patients reviewed, 22 tested positive for the MDA5 antibody, comprising of thirteen adults and nine pediatric patients. Myositis was significantly more common in pediatric patients than in adult patients (p = 0.002). RP-ILD was more frequently observed in adult patients of African ancestry (including both Black Hispanic and Black non-Hispanic individuals) (p = 0.04). There was a significant association noted between Raynaud’s phenomenon and ILD (p = 0.02). The overall mortality rate of 13.6% was more favorable than the previously reported rates of 40–60%. Conclusion This study enhances our understanding of the disease by emphasizing potential racial and demographic variations, as well as delineating the similarities and differences between adult and pediatric populations. |
| format | Article |
| id | doaj-art-c07bad99c5234cb59703516eb41679cd |
| institution | Kabale University |
| issn | 2520-1026 |
| language | English |
| publishDate | 2025-01-01 |
| publisher | BMC |
| record_format | Article |
| series | BMC Rheumatology |
| spelling | doaj-art-c07bad99c5234cb59703516eb41679cd2025-01-12T12:44:03ZengBMCBMC Rheumatology2520-10262025-01-01911710.1186/s41927-025-00455-5Antimelanoma differentiation-associated gene 5 (anti-MDA5) antibody-positive dermatomyositis: clinical features and outcomes in a racially diverse patient cohortSai K. Koyoda0Fatema Ezzy1Dawn Wahezi2Anand Kumthekar3Xianhong Xie4Clement E. Tagoe5Bibi Ayesha6Montefiore Medical Center, Albert Einstein College of Medicine, RheumatologyJacobi Medical Center, Department of Internal MedicineDepartment of Pediatric Rheumatology, Children’s Hospital at MontefioreMontefiore Medical Center, Albert Einstein College of Medicine, RheumatologyDepartment of Epidemiology and Population Health, Albert Einstein College of MedicineMontefiore Medical Center, Albert Einstein College of Medicine, RheumatologyMontefiore Medical Center, Albert Einstein College of Medicine, RheumatologyAbstract Background The anti-melanoma differentiation-associated gene 5 (anti-MDA5) antibody-positive dermatomyositis is known for its association with rapidly progressive interstitial lung disease (RP-ILD) and ulcerative skin lesions, often presenting with or without muscle involvement. The aim of this study was to identify distinct clinical and laboratory features that could be used to evaluate disease progression in an ethnically diverse cohort of anti-MDA5 dermatomyositis patients at a U.S. academic center. Methods A retrospective chart review was conducted on dermatomyositis patients hospitalized at our institution between January 2014 and June 2023. The data were analyzed via Fischer’s exact test and a t test. Results Among the 195 dermatomyositis patients reviewed, 22 tested positive for the MDA5 antibody, comprising of thirteen adults and nine pediatric patients. Myositis was significantly more common in pediatric patients than in adult patients (p = 0.002). RP-ILD was more frequently observed in adult patients of African ancestry (including both Black Hispanic and Black non-Hispanic individuals) (p = 0.04). There was a significant association noted between Raynaud’s phenomenon and ILD (p = 0.02). The overall mortality rate of 13.6% was more favorable than the previously reported rates of 40–60%. Conclusion This study enhances our understanding of the disease by emphasizing potential racial and demographic variations, as well as delineating the similarities and differences between adult and pediatric populations.https://doi.org/10.1186/s41927-025-00455-5MDA5 autoantibodyDermatomyositisRace & ethnicityInterstitial lung disease |
| spellingShingle | Sai K. Koyoda Fatema Ezzy Dawn Wahezi Anand Kumthekar Xianhong Xie Clement E. Tagoe Bibi Ayesha Antimelanoma differentiation-associated gene 5 (anti-MDA5) antibody-positive dermatomyositis: clinical features and outcomes in a racially diverse patient cohort BMC Rheumatology MDA5 autoantibody Dermatomyositis Race & ethnicity Interstitial lung disease |
| title | Antimelanoma differentiation-associated gene 5 (anti-MDA5) antibody-positive dermatomyositis: clinical features and outcomes in a racially diverse patient cohort |
| title_full | Antimelanoma differentiation-associated gene 5 (anti-MDA5) antibody-positive dermatomyositis: clinical features and outcomes in a racially diverse patient cohort |
| title_fullStr | Antimelanoma differentiation-associated gene 5 (anti-MDA5) antibody-positive dermatomyositis: clinical features and outcomes in a racially diverse patient cohort |
| title_full_unstemmed | Antimelanoma differentiation-associated gene 5 (anti-MDA5) antibody-positive dermatomyositis: clinical features and outcomes in a racially diverse patient cohort |
| title_short | Antimelanoma differentiation-associated gene 5 (anti-MDA5) antibody-positive dermatomyositis: clinical features and outcomes in a racially diverse patient cohort |
| title_sort | antimelanoma differentiation associated gene 5 anti mda5 antibody positive dermatomyositis clinical features and outcomes in a racially diverse patient cohort |
| topic | MDA5 autoantibody Dermatomyositis Race & ethnicity Interstitial lung disease |
| url | https://doi.org/10.1186/s41927-025-00455-5 |
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