Macrophage Activation Syndrome Associated with Adult-onset Still's Disease Successfully Treated with Plasma Exchange and Canakinumab.
Wataru Kiyohara, Yuya Fujita, Muneyuki Hatta et al.
✦ AI-curated · Sources linked
Case in brief
A 20-year-old Japanese woman with adult-onset Still’s disease developed macrophage activation syndrome despite prednisolone and tocilizumab. Findings included hyperferritinemia, thrombocytopenia, hypertriglyceridemia, and liver dysfunction. Steroid pulse therapy, cyclosporine A, and plasma exchange improved her condition, but diabetes made glycemic control difficult during high-dose steroid treatment. Canakinumab was then started, allowing prednisolone to be tapered to 10 mg within one month without relapse.
What made this case unusual
Diabetes complicated high-dose steroid treatment, prompting canakinumab after plasma exchange to facilitate rapid steroid tapering without relapse.
Diagnostic / clinical pearl
In this case, plasma exchange and canakinumab were used sequentially for severe AOSD-associated MAS when limiting steroid exposure was important.