A full read systematic review article examined treatment options for difficult to manage systemic juvenile idiopathic arthritis, or Still’s disease. It classified these refractory disease patients as those with either (1) persistent arthritis, (2) recurrent or difficult-to-treat macrophage activation syndrome (MAS), or (3) interstitial lung disease. 

The review included 30 articles – 1 pilot study, 8 case series 21 case reports, but no controlled rials. There were few takeaways: 

• There were positive results in a pilot study of emapalumab (IFN-gamma inhibitor) for the treatment of refractory MAS
• Beneficial results with combination biologics and Janus kinase (JAK) inhibitors for refractory Still’s arthritis. While there are reports of successful management with allogenic stem cell transplantation, this comes with significant risks.

There is very limited data regarding treatment strategies for refractory Still’s, but there is limited evidence to suggest the use of emapalumab, JAK inhibitors, rituximab and combination biologics.

Further study and innovative trial designs are needed to define the optimal approach to these challenging subgroups of Still’s disease patients.