Gastrointestinal involvement in dermatomyositis: multicentric retrospective cohort study
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.
Authors
- Luc Mouthon (ORCID: https://orcid.org/0000-0002-7021-9492)
- Eric HACHULLA (ORCID: https://orcid.org/0000-0001-7432-847X)
- Yves Allenbach (ORCID: https://orcid.org/0000-0002-3185-7993)
- Olivier Benvéniste (ORCID: https://orcid.org/0000-0002-1167-5797)
- Delphine Larivière
- Alain Meyer (ORCID: https://orcid.org/0000-0003-0893-6041)
- D. Staumont‐Sallé (ORCID: https://orcid.org/0000-0002-4780-4212)
- Nicolas Limal
- Laure Gallay (ORCID: https://orcid.org/0000-0002-7020-1954)
- Laurent Gilardin (ORCID: https://orcid.org/0000-0001-9212-0859)
- Sarah Léonard-Louis (ORCID: https://orcid.org/0000-0002-2365-7932)
- C. Fite (ORCID: https://orcid.org/0009-0006-9436-5511)
- Valérie Jooste (ORCID: https://orcid.org/0000-0002-9902-0700)
- Nathalie Costedoat-Chalumeau (ORCID: https://orcid.org/0000-0002-1555-9021)
- Solal Bellaiche
- Mohamed-Yacine Khitri (ORCID: https://orcid.org/0000-0002-2090-5750)
- Anne‐Marie Bouvier (ORCID: https://orcid.org/0000-0001-9070-1027)
- A Bardier
- Benoît Gachet (ORCID: https://orcid.org/0009-0007-3001-3258)
- Benjamin Terrier (ORCID: https://orcid.org/0000-0001-6612-7336)
- Stephane Vinzio
- Jean Luc Charuel
- Camille Rasmussen
- Yoann Crabol
- Juliette Avci
- S. Tayb-Boulahfa
- Jean Baptiste Noury (ORCID: https://orcid.org/0009-0002-3917-9186)
- Orane Demuynck (ORCID: https://orcid.org/0009-0008-2142-6461)
Institutions
- Université Libre de Bruxelles (BE)
- Inserm (FR)
- Université de Bourgogne (FR)
- Université Paris-Est Créteil (FR)
- Université Paris Cité (FR)
- Aix-Marseille Université (FR)
- Université de Lille (FR)
- Centre Hospitalier Universitaire de Lille (FR)
- Sorbonne Université (FR)
- Lille’s Cardiology Hospital (FR)
- Hôpital Cochin (FR)
- Assistance Publique – Hôpitaux de Paris (FR)
- Hospices Civils de Lyon (FR)
- Centre hospitalier Bretagne Atlantique (FR)
- Laboratoire National de Référence (MA)
- Groupe Hospitalier Mutualiste de Grenoble (FR)
- Institut de Myologie (FR)
- Centre Hospitalier de Tourcoing (FR)
- CHU Dijon Bourgogne (FR)
- Hôpital Saint Joseph (FR)
- Pitié-Salpêtrière Hospital (FR)
- Laboratoire des Sciences et Techniques de l’Information de la Communication et de la Connaissance (FR)
- Centre Hospitalier Régional Universitaire de Brest (FR)
- Hôpitaux Universitaires de Strasbourg (FR)
- Hôpital Jean-Verdier (FR)
- Hôpital Foch (FR)
Publication Details
- Journal
- Lara D. Veeken
- Published
- 2026-06-17
- DOI
- https://doi.org/10.1093/rheumatology/keag282
- Primary Topic
- Inflammatory Myopathies and Dermatomyositis
- Type
- article
- Field-Weighted Citation Impact
- 0.00