The place of JAK inhibitors in systemic juvenile idiopathic arthritis with lung disease: French experience
Authors' conclusion
JAKis offer another therapeutic option for patients with SJIA-LD.
Study context
- Population
- Patients with sJIA with associated lung disease (SJIA-LD), French paediatric rheumatology centres
- Participants
- 8 patients
- Outcome
- Disease control with JAKi
Methodological detailsmedical records review, multicentre
- Medical records review
- Multicentre
Main finding
As reported in the abstract
Complete disease control
4
Steroids tapered
4
Steroids stopped
2
Observational data: associations, not effects.
Further findings
Status at last follow-up
- Died from severe macrophage activation syndrome (MAS)
- 1
- Underwent haematopoietic stem cell transplantation
- 2
- Complete response (two of them free of steroids)
- 4
- Partial response with janus kinase inhibitors (JAKis)
- 1
Lung response vs disease duration
- Lung response to JAKi not clearly linked to disease duration
Safety
| Outcome | Values |
|---|---|
| Macrophage activation syndrome shortly after anti-IL-1s stopped when JAKis introduced | JAKi 3 events |
| Other serious side effects (viral reactivation-EBV, BK virus, cytopenia) | JAKi 2 events |
Original publication
The place of JAK inhibitors in systemic juvenile idiopathic arthritis with lung disease: French experience. Rheumatology (Oxford). Published 24 October 2024. doi:10.
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