Double-filtration plasmapheresis for resolution of corticosteroid resistant adult onset Still's disease.

A 45-year-old Japanese male was diagnosed with adult onset Still's disease (AOSD). High-dose corticosteroid initially resolved the illness; however, high fever, maculopalpular rashes, arthralgia, and acute pericarditis rapidly recurred, and were followed by a somnolent state without focal signs. A diagnosis of corticosteroid resistant, severe, recurrent AOSD was made, and double-filtration plasmapheresis (DFPP) was performed immediately. The somnolent state began to resolve during the first plasmapheresis procedure, and the other symptoms resolved shortly thereafter. DFPP theoretically removes monocyte-activating cytokines, such as monocyte colony-stimulating factor (M-CSF) from the circulation, and therefore may prove to be an effective treatment for corticosteroid resistant, rapidly developing cases of AOSD.