Myositis-specific autoantibody subtypes are associated with response to Janus kinase inhibitors in patients with juvenile dermatomyositis
Authors' conclusion
JAKi induced CID in the majority of new-onset and refractory JDM patients, except in a subset of patients with refractory TIF1γ-positive JDM. Overall tolerance was acceptable.
Study context
- Population
- Patients with new-onset and refractory JDM treated with Janus kinase inhibitors (JAKi) for at least 6 months, single centre
- Participants
- 39 patients
- Outcome
- Proportion achieving CID (PRINTO criteria and Skin Disease Activity Score)
- Follow-up
- Within 6 months after initiation of JAKi
Methodological detailssingle-centre
- Single-centre
Main finding
As reported in the abstract
Partial or complete CID (PRINTO criteria and Skin Disease Activity Score) · 6 months
Partial or complete clinically inactive disease (CID) achieved after 6 months of JAKi treatment
32 of 39
82%
Observational data: associations, not effects.
Further findings
Mean steroid dose in responders · 6 months
- Mean steroid dose decreased from 1 to 0 mg/kg/day; all other medications withdrawn
- P = 0.001
Factor at JDM onset associated with absence of CID (multivariable analysis)
- Anti-TIF1γ Abs the only factor associated with absence of CID
- P < 0.001
Type 1 IFN score and serum IFN-α, diagnosis to 6-month follow-up
- Significant decrease in median Type 1 IFN score and serum IFN-α observed only in patients with CID
Safety
| Outcome | Values |
|---|---|
| JAKi-related infectionsIncluding five herpes zoster infections | JAKi 9 events |
| JAKi-related weight gain | JAKi 3 events |
Original publication
Myositis-specific autoantibody subtypes are associated with response to Janus kinase inhibitors in patients with juvenile dermatomyositis. Rheumatology (Oxford). Published 24 May 2025. doi:10.
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