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Clinical characteristics and long-term prognosis of anti-MDA5-positive dermatomyositis: a comparative study across
Fang Dong1, Panpan Zhang1, Wenlu Hu1
1Clinical Immunology and Rheumatology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou University, Zhengzhou, Henan Province, China.
Objectives:
Research 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.
Methods:
We 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.
Results:
In 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.
Conclusions:
Clinical 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.
Insights
Age significantly impacts clinical presentation and prognosis in anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis (anti-MDA5+ DM). Older patients (≥60 years) face higher mortality risks, particularly with Pneumocystis jirovecii pneumonia (PJP) infection.
Area of Science:
- Rheumatology
- Immunology
- Dermatology
Background:
- Limited research exists on age-related clinical differences and prognosis in anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis (anti-MDA5+ DM).
- Understanding these variations is crucial for tailoring treatment and improving patient outcomes.
Purpose of the Study:
- To investigate the clinical manifestations and long-term mortality of anti-MDA5+ DM patients across different age groups.
- To identify age-specific risk factors and develop a prognostic model for mortality.
Main Methods:
- A cohort of 318 newly diagnosed anti-MDA5+ DM patients was analyzed.
- Cochran-Armitage test for trend (CATT) assessed age-related clinical characteristic changes.
- Cox regression and a nomogram model were used for mortality risk stratification.
Main Results:
- Clinical features like cough, Pneumocystis jirovecii pneumonia (PJP), dyspnea, and rapidly progressive interstitial lung disease (RP-ILD) increased with age.
- Rash and arthralgia prevalence decreased in older age groups.
- A novel nomogram incorporating age and PJP infection demonstrated independent and combined effects on mortality.
Conclusions:
- Clinical and laboratory findings in anti-MDA5+ DM vary significantly by age.
- Advanced age (≥60 years) and PJP infection are key indicators of poor prognosis.
- The developed nomogram aids in early risk stratification for improved clinical decision-making.
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