Improvement in Disease Activity in Refractory Juvenile Dermatomyositis Following Abatacept Therapy
Authors' conclusion
This open-label study demonstrated that abatacept may be beneficial for patients with treatment-refractory juvenile DM.
Study context
- Population
- Patients ≥7 years of age with refractory JDM and moderate disease activity
- Participants
- 10 patients
- Outcome
- International Myositis Assessment and Clinical Studies (IMACS) Definition Of Improvement (DOI)
Methodological details24-week, blinded MRI assessment
- 24-week
- Blinded MRI assessment
Main finding
As reported in the abstract
IMACS DOI
Patients achieving Definition Of Improvement (DOI) at week 12
5
Patients achieving DOI at week 24
9
Secondary outcomes
Improvement by 2016 American College of Rheumatology (ACR)/EULAR response criteria for JDM (among DOI achievers) · week 24
- Minimal improvement
- 2
- Moderate improvement
- 4
- Major improvement
- 3
Core set activity measures · weeks 12 and 24
- Improvements from baseline in all CSMs except muscle enzymes
Daily GC dose · week 24
- Mean baseline dose (mg)
- 16.7
- Mean dose at week 24 (mg)
- 10.2
- P
- P = 0.002
MRI muscle edema score · week 24
- Mean baseline score
- 5.3
- Score at week 24
- 2.3
- P
- P = 0.01
Interferon (IFN)-regulated gene scores and galectin-9 expression · week 24
- Patients with down-trending scores
- 6
- Decreases in IFN-regulated gene scores, IP-10, galectin-9 and IL-2 correlated with improvements in disease activity and MRI muscle edema
Safety
- Grade 2 or 3 treatment-emergent adverse events observed
- 11
Original publication
Improvement in Disease Activity in Refractory Juvenile Dermatomyositis Following Abatacept Therapy. Arthritis Rheumatol. Published 7 June 2023. doi:10.
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