Gastrointestinal involvement in dermatomyositis: multicentric retrospective cohort study

Area of Science:

• Rheumatology
• Gastroenterology
• Immunology

Background:

• Gastrointestinal (GI) involvement in myositis, a severe extra-muscular manifestation, can have life-threatening outcomes but remains poorly understood.
• Understanding the prevalence and characteristics of GI involvement in dermatomyositis (DM) is crucial for patient management.

Purpose of the Study:

• To evaluate the prevalence of GI involvement in DM.
• To characterize GI involvement specifically in anti-NXP2 antibody-positive DM patients.

Main Methods:

• Assessed GI involvement prevalence across DM subgroups in a monocentric cohort (n=119).
• Investigated GI involvement in a multicentric cohort of anti-NXP2 DM patients (n=48).
• Defined severe GI involvement as requiring surgery and/or ICU admission.

Main Results:

• Severe GI involvement was more frequent in anti-NXP2 DM (18%) compared to other DM subgroups (6%).
• In the anti-NXP2 cohort, 12.5% experienced severe GI involvement, including perforations and hemorrhages.
• Anti-NXP2 patients with severe GI involvement were younger (median 24.5 years) and more likely to have facial edema (83%) at diagnosis.

Conclusions:

• Severe GI involvement is a significant, potentially life-threatening complication in DM, primarily linked to the anti-NXP2 antibody.
• In anti-NXP2 DM, younger age and facial edema at diagnosis are associated with a higher risk of severe GI involvement.