Pediatric rheumatology

Clinical features, triggers, and risk factors of severe gastrointestinal involvement in anti-NXP2-positive juvenile dermatomyositis: a retrospective cohort study

Pediatr Rheumatol Online JPublished Retrospective cohort study

Authors' conclusion

Infection may be an important trigger. Risk factors for GI complications include severe JDM at onset, malnutrition, skin ulceration, edema, low CMAS score, high CAT-A score, lymphocytopenia, anemia, hypoalbuminemia and elevated ESR.

Study context

Population
Anti-NXP2-positive JDM patients
Participants
57 patients
Exposure
Anti-NXP2-JDM with severe gastrointestinal (GI) complications vs without GI involvement
  • GIn = 19
  • Non-GIn = 38reference
Outcome
Clinical characteristics, triggers and risk factors for GI complications
Methodological detailsmultivariate analysis, Kaplan-Meier analysis
  • Multivariate analysis
  • Kaplan-Meier analysis

Main finding

As reported in the abstract

Risk factors for GI complications (multivariate analysis)
  • Independent risk factors: severe JDM at onset, infection, malnutrition, CAT-A score, skin ulcer, edema and ESR
  • Protective factors: lymphocyte count, proximal muscle strength, CMAS score, hemoglobin and albumin

Observational data: associations, not effects.

Further findings

Deaths

P = 0.002

Prodromal infections (potential triggers) in GI group
84% · 16 of 19
GI group characteristics
5.83 · mean · years

Prodromal infections (potential triggers) in GI group

All severe GI involvement occurred during active JDM

GI group characteristics

Abdominal pain the most common initial symptom

Survival (Kaplan-Meier)

Significantly different survival rates between groups

Original publication

Clinical features, triggers, and risk factors of severe gastrointestinal involvement in anti-NXP2-positive juvenile dermatomyositis: a retrospective cohort study. Pediatr Rheumatol Online J. Published 22 May 2026. doi:10.1186/s12969-026-01219-2

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