Time-dependent changes in RPILD and mortality risk in anti-MDA5+ DM patients: a cohort study of 272 cases in China
Hanxiao You1, Lei Wang1, Jiajia Wang1
1Department of Rheumatology, The First Affiliated Hospital of Nanjing Medical University, Nanjing.
Objectives:
Anti-melanoma differentiation-associated gene 5 positive (anti-MDA5+) DM has a close relationship with rapidly progressive interstitial lung disease (RPILD) and is associated with high mortality. However, data regarding the time-dependent risk of RPILD and deaths during disease progression are limited. We conducted this study to investigate whether the risk of RPILD and death were time-dependent or not in anti-MDA5+ DM.
Methods:
We assessed a cohort of 272 patients with anti-MDA5+ DM. The clinical characteristics of patients with anti-MDA5+ were collected, and COX regression was used to analyse independent risk factors for RPILD and death. We also described changes in risk of RPILD and death over time and their potential clinical implications.
Results:
There were 272 anti-MDA5+ DM patients enrolled in this study. According to the multivariate cox regression analysis, short disease course, high CRP level, anti-Ro52 positive and anti-MDA5 titre (++∼+++) were independent risk factors of RPILD. High creatine kinase level, high CRP level and RPILD were independent risk factors for death, and >90% RPILD and 84% mortality occurred in the first 6 months after disease onset. Notably, the first 3 months is a particularly high-risk period, with 50% of RPILD and 46% of deaths occurring. Hazards regarding RPILD and mortality diminished over time during a median follow-up of 12 months.
Conclusion:
These results suggest significant, time-dependent changes in RPILD and mortality risk in anti-MDA5+ DM patients, providing a cut-off time window to estimate disease progression and poor prognosis.
Insights
The risk of rapidly progressive interstitial lung disease (RPILD) and death in dermatomyositis with anti-melanoma differentiation-associated gene 5 positive antibodies (anti-MDA5+ DM) significantly decreases over time. The initial 3-6 months post-onset represent the highest risk period for these complications.
Area of Science:
- Rheumatology
- Pulmonology
- Immunology
Background:
- Anti-melanoma differentiation-associated gene 5 positive dermatomyositis (anti-MDA5+ DM) is strongly linked to rapidly progressive interstitial lung disease (RPILD) and carries a high mortality rate.
- Limited data exists on the temporal dynamics of RPILD and mortality risk during the course of anti-MDA5+ DM.
Purpose of the Study:
- To investigate the time-dependent nature of RPILD and mortality risk in patients with anti-MDA5+ DM.
- To identify key risk factors and high-risk periods for RPILD and death in this patient cohort.
Main Methods:
- A cohort of 272 patients with anti-MDA5+ DM was analyzed.
- Cox regression models were employed to identify independent risk factors for RPILD and death.
- The temporal trends of RPILD and mortality risk were assessed over time.
Main Results:
- Short disease duration, elevated CRP, anti-Ro52 positivity, and high anti-MDA5 titres were identified as independent risk factors for RPILD.
- High creatine kinase, elevated CRP, and the presence of RPILD were independent risk factors for mortality.
- Over 90% of RPILD cases and 84% of deaths occurred within the first 6 months, with a peak risk in the first 3 months.
- RPILD and mortality risks demonstrated a diminishing trend over the median 12-month follow-up period.
Conclusions:
- The risk of RPILD and death in anti-MDA5+ DM is significantly time-dependent, with a pronounced high-risk period early in the disease course.
- Identifying this early high-risk window is crucial for timely intervention and management to improve patient prognosis.
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