Severe gastrointestinal involvements in patients with adult dermatomyositis with anti-NXP2 antibody
An erratum to this article was published (RMD Open, 30 Jan 2024 ↗)
Authors' conclusion
GI involvement is a unique complication in patients with adult NXP2+DM.
Study context
- Population
- Adult patients with anti-NXP2 DM (NXP2+DM), single hospital, 2012 to 202210 with gastrointestinal (GI) involvements
- Participants
- 56 patients
- Exposure
- NXP2+DM with GI involvements vs without
- GI involvementsn = 10
- Outcome
- GI involvement in adult NXP2+DM
Methodological detailssingle-centre, RNA sequencing of intestinal samples vs healthy controls data set
- Single-centre
- RNA sequencing of intestinal samples vs healthy controls data set
Main finding
As reported in the abstract
- Patients with GI involvements
- 10 of 56
- Median time lag from NXP2+DM diagnosis to GI involvement (abdominal pain and melena as initial manifestations), when myositis largely subsided
- 10-month
- GI perforation within weeks
- 8 of 10
- Patients undergoing surgical interventions (eight interventions)
- 5
- Short-term mortality
- 4
Observational data: associations, not effects.
Further findings
Extramuscular manifestations
- NXP2+DM with GI involvements presented with more extramuscular systemic manifestations such as ILD and subcutaneous calcinosis
GI pathology
- Vasculitis/vasculopathy with high MxA expression, intestinal smooth muscle necrosis and serosal calcinosis
Gene expression profile
- Type-I interferon activation validated; epithelial mesenchymal transition and focal adhesion pathway may also contribute
Treatment
- Vedolizumab (anti-α4β7-integrin monoclonal antibody) exhibited promising therapeutic signals
Original publication
Severe gastrointestinal involvements in patients with adult dermatomyositis with anti-NXP2 antibody. RMD Open. Published 10 January 2024. doi:10.
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