Thrombotic microangiopathy in a patient with adult onset Still's disease

We report a case of a 23-year-old man with adult onset Still's disease (AOSD) developing a rare, life-threatening complication of thrombotic microangiopathy (TMA). While the AOSD was in an active phase, our patient first developed hemolytic uremia syndrome, soon followed by convulsions, sudden loss of vision, and thrombotic angiopathy of retinal vessels. After immediate and aggressive treatment with high dose prednisolone and 18 courses of plasmapheresis, he recovered from this severe complication. We think that the occurrence of TMA in AOSD may not be coincidental, although more reports are needed to support this. Early recognition and aggressive immunotherapy can allow patients with AOSD to completely recover from this life-threatening complication.