ArticleOrphanet journal of rare diseases2026
Clinical characteristics and long-term prognosis of anti-MDA5-positive dermatomyositis: a comparative study across age groups.
Article in Orphanet journal of rare diseases, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
objectivesResearch focused on clinical differences and long-term prognosis in anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis (anti-MDA5+ DM) patients across age groups remains limited. This study aimed to explore the differences in the clinical manifestations and long-term mortality of anti-MDA5+ DM patients across age groups.
methodsWe included 318 newly diagnosed anti-MDA5+ DM patients, recruited from June 2018 to January 2024. The median follow-up time was 22.5 months (4.5-36 months). The Cochran-Armitage test for trend (CATT) was employed to assess the statistical significance of changes in the proportion of clinical characteristics across different age groups. Cox regression analysis and a nomogram model were developed to stratify the risk associated with mortality.
resultsIn the cohort of 318 patients, 123 (38.7%) were aged < 50 years, 124 (39.0%) were aged 50-59 years, and 71 (22.3%) were aged ≥ 60 years. Clinical manifestations and comorbidities such as cough, Pneumocystis jirovecii pneumonia (PJP), dyspnea, and rapidly progressive interstitial lung disease (RP-ILD) increased with age, while rash and arthralgia decreased. PJP was a major factor in poor prognosis, especially among older patients who were more susceptible to infection. The nomogram, the first prognostic model incorporating both age and PJP infection in anti-MDA5+ DM, demonstrated its independent and combined effects on mortality and enabled early risk stratification, providing a valuable tool for clinical decision-making.
conclusionsClinical manifestations and laboratory parameters varied in anti-MDA5+ DM patients across different age groups. Advanced age and PJP are major factors associated with poor prognosis, with patients aged ≥ 60 years showing the highest mortality and being predominat in the high-risk group.
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