Autoimmunity reviews · 2026
Subtype-specific mortality in idiopathic inflammatory myopathies: A hospital-based cohort.
СтатьяОбзорНазвание и аннотация: Перевод в очереди
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Larrauffie Aurore, Bost Chloé, Barrier Claire, Villeneuve Thomas, Acket Blandine, Alric Laurent, Bonnet Delphine, Cintas Pascal, Constantin Arnaud, Cougoul Pierre, Chaves Sébastien De Almeida, Uro-Coste Emmanuelle, Guille Marie, Maquet Julien, Moulis Guillaume, Mourguet Morgane, Delpuech Antoine, Piel-Julian Marie Léa, Prévot Grégoire, Sailler Laurent, Biteau Mélanie, Zordan Jérémy, Faruch Marie, Pugnet Grégory
Аннотация
INTRODUCTION: The aim of our study is to compare mortality rates, causes of death and associated risk factors for idiopathic inflammatory myopathies (IIM) and their various subtypes in a hospital-based cohort, specially IMNM (immune-mediated necrotizing myopathy).
MATERIALS AND METHODS: This retrospective study was conducted using the MIIRTALITY cohort, including patients followed for IIM from 2010 to 2022. They were then categorized into five subtypes: dermatomyositis (DM), polymyositis (PM), antisynthetase syndrome (ASS), immune-mediated necrotizing myopathy (IMNM) and overlap myositis (OM). Survival analysis based on IIM subtypes was performed using the Kaplan-Meier method. Cox proportional hazards models were used to determine baseline variables associated with mortality.
RESULTS: During the study period, 33 of the 225 patients enrolled in this study died. IMNM have a mortality rate of 1.7 per 100 person-year. The poorest rate of survival was found in patients with DM (HR 3.32; 95% CI 1.61-6.86; p = 0.001), and the lowest mortality rate, when compared to the rest of IIM, was found in patients with ASS (HR 0.41; 95% CI 0.19-0.88). The presence of malignancy was associated with a markedly increased risk of death (HR 12.2; 95% CI 3.09-48.13; p < 0.001). Joint involvement and classification as a non-DM IIM subtype were associated with a reduced risk of death (HR 0.37; 95% CI 0.18-0.76; p = 0.006 and HR 0.06; 95% CI 0.01-0.36; p = 0.002), respectively.
CONCLUSION: Dermatomyositis had the highest mortality among IIM subtypes in our hospital-based cohort, ASS was associated with better survival, highlighting the importance of differentiating IIM subtypes so as to personalize treatment and follow-up strategies. In our hospital-based cohort, IMNM mortality was not higher than that of others subtypes. These findings underscore the importance of distinguishing IIM subtypes to personalize treatment and follow-up strategies.
MATERIALS AND METHODS: This retrospective study was conducted using the MIIRTALITY cohort, including patients followed for IIM from 2010 to 2022. They were then categorized into five subtypes: dermatomyositis (DM), polymyositis (PM), antisynthetase syndrome (ASS), immune-mediated necrotizing myopathy (IMNM) and overlap myositis (OM). Survival analysis based on IIM subtypes was performed using the Kaplan-Meier method. Cox proportional hazards models were used to determine baseline variables associated with mortality.
RESULTS: During the study period, 33 of the 225 patients enrolled in this study died. IMNM have a mortality rate of 1.7 per 100 person-year. The poorest rate of survival was found in patients with DM (HR 3.32; 95% CI 1.61-6.86; p = 0.001), and the lowest mortality rate, when compared to the rest of IIM, was found in patients with ASS (HR 0.41; 95% CI 0.19-0.88). The presence of malignancy was associated with a markedly increased risk of death (HR 12.2; 95% CI 3.09-48.13; p < 0.001). Joint involvement and classification as a non-DM IIM subtype were associated with a reduced risk of death (HR 0.37; 95% CI 0.18-0.76; p = 0.006 and HR 0.06; 95% CI 0.01-0.36; p = 0.002), respectively.
CONCLUSION: Dermatomyositis had the highest mortality among IIM subtypes in our hospital-based cohort, ASS was associated with better survival, highlighting the importance of differentiating IIM subtypes so as to personalize treatment and follow-up strategies. In our hospital-based cohort, IMNM mortality was not higher than that of others subtypes. These findings underscore the importance of distinguishing IIM subtypes to personalize treatment and follow-up strategies.