Gastrointestinal involvement in dermatomyositis: multicentric retrospective cohort study
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ID: 317606
2026
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Abstract
Abstract Objectives Gastrointestinal (GI) involvement in myositis is a severe extra muscular manifestation with potentially life-threatening outcomes. However, it remains poorly characterized. We aimed to evaluate the prevalence of GI involvement in dermatomyositis (DM) and to describe its characteristics in anti-NXP2 DM. Methods The prevalence of GI involvement across DM subgroups was assessed in a retrospective monocentric DM cohort (n = 119). It was further investigated in a retrospective multicentric cohort of anti-NXP2 DM (n = 48). GI involvement (defined as any symptomatic GI lesion, excluding isolated dysphagia) was considered severe when requiring surgery and/or intensive care unit admission. Results In the monocentric DM cohort, severe GI involvement was observed in 18% of anti-NXP2 patients and 6% of anti-MDA5 patients. Severe GI involvement was significantly more frequent in anti-NXP2 patients compared with other DM subgroups (hazard ratio 8.85 [95% CI 1.62–48.35]). In the multicentric anti-NXP2 cohort, severe GI involvement (including four digestive perforations and three digestive haemorrhages) occurred in 12.5% of patients. Anti-NXP2 patients with severe GI involvement were significantly younger (median age 24.5 vs 42.5 years; p< 0.01) and more frequently presented with facial oedema at diagnosis (83% vs 33%; p= 0.03). At the time of GI involvement, 78% of anti-NXP2 patients had concomitant skin and/or muscle disease activity. No deaths were reported. Conclusion Severe GI involvement represents a life-threatening complication in DM and is mainly associated with anti-NXP2 antibody. In anti-NXP2 DM, young age and facial oedema are associated with severe GI involvement.
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| Authors | Orane Demuynck, Camille Rasmussen, Mohamed-Yacine Khitri, Alain Meyer, É. Hachulla, Delphine Larivière, D. Staumont‐Sallé, N. Costedoat‐Chalumeau, Luc Mouthon, Benjamin Terrier, Nicolas Limal, Jean Baptiste Noury, C. Fite, Yoann Crabol, Laurent Gilardin, S. Vinzio, Benoît Gachet, Solal Bellaiche, Anne‐Marie Bouvier, Sarah Léonard-Louis, A Bardier, Juliette Avci, Valérie Jooste, Jean Luc Charuel, S. Tayb-Boulahfa, Laure Gallay, Olivier Benvéniste, Yves Allenbach |
| Journal | Lara D. Veeken |
| Year | 2026 |
| DOI |
10.1093/rheumatology/keag282
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| URL | |
| Keywords | Keywords not found |
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