A small retrospective study suggests that patients with difficult to treat adult-onset Still’s disease (AOSD) or sytemic juvenile idiopathic arthritis (sJIA) may respond well to JAK inhibitor (JAKi) agents – presumable by blocking pro-inflammatory cytokines, notably IL-6 and IFN.
Patients with sJIA or AOSD were gathered based on a national survey conducted in the departments of rheumatology, paediatric rheumatology and internal medicine of French hospitals.
A total of 9 patients (7 AOSD, 2 sJIA) with an inadequate response to corticosteroids, DMARDs or biologics. Jak inhibitor use included Baricitinib (5 patients), ruxolitinib in 2, tofacitinib in 2, and upadacitinib in 1. A JAKi was associated with anakinra and corticosteroids in one patient, and with methotrexate, anakinra and corticosteroids in another.
With a median follow-up of 16 months, 2/9 were in complete remission, 3/9 partial response and 4/9 treatment failure. At the last visit, corticosteroids could be decreased but not stopped.
These observations say that JAKi agents may be an option for some difficult-to-treat Still’s disease, but an unmet need remains for others.