Association of NXP2 autoantibodies with a more severe clinical phenotype of juvenile dermatomyositis
Authors' conclusion
The NXP2+ JDM patients experience more severe muscle damage, systemic complications, and higher relapse risks.
Study context
- Population
- Patients with juvenile dermatomyositis (JDM) positive for anti-NXP2, anti-MDA5 or anti-TIF1γ, and NXP2+ adult DM patients
- Participants
- 18 / 12 / 12 / 20
- Exposure
- NXP2+ JDM vs MDA5+ JDM, TIF1γ+ JDM and NXP2+ adult DM
- NXP2+ JDMn = 18
- MDA5+ JDMn = 12
- TIF1γ+ JDMn = 12
- NXP2+ adult DMn = 20
- Outcome
- Serum creatine kinase levels
Methodological detailscomparative, 3-year follow-up, repeated measures analysis, Kaplan-Meier and Cox regression
- Comparative
- 3-year follow-up
- Repeated measures analysis
- Kaplan-Meier and Cox regression
Main finding
As reported in the abstract
NXP2+ JDM
3792
MDA5+ JDM
180
NXP2+ adult DM
437.5
TIF1γ+ JDM
189
NXP2+ JDM vs MDA5+ JDM: P < 0.001 · NXP2+ JDM vs NXP2+ adult DM: P = 0.003 · NXP2+ JDM vs TIF1γ+ JDM: P < 0.001
Observational data: associations, not effects.
Further findings
| Outcome | NXP2+ JDM | MDA5+ JDM | TIF1γ+ JDM | P value |
|---|---|---|---|---|
| CMAS scoresNXP2+ JDM vs MDA5+ JDM | 31.33 | 43.25 | 42.67 | P < 0.001 |
| CMAS scoresNXP2+ JDM vs TIF1γ+ JDM | P = 0.004 |
Clinical complications
- NXP2+ JDM presented with a high frequency of macrophage activation syndrome (MAS), myocardial damage, dysphagia and calcinosis
Longitudinal muscle damage (repeated measures) · throughout the disease course
- NXP2+ JDM had more severe muscle damage than MDA5+ JDM and TIF1γ+ JDM
- Significant Group × Time interaction effects on creatine kinase (CK) in JDM
Relapse risk · 3-year follow-up
- Higher relapse risk in NXP2+ JDM vs MDA5+ JDM, TIF1γ+ JDM and NXP2+ adult DM
Original publication
Association of NXP2 autoantibodies with a more severe clinical phenotype of juvenile dermatomyositis. Clin Exp Rheumatol. Published 9 April 2025. doi:10.
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