engleski

Thrombotic microangiopathy in adult onset Still's disease: case report and review of the literature

Summary: Coexistence of thrombotic microangiopathy and adult onset Still's disease (AOSD) is extremely rare. There is increasing evidence that this association could be more than just coincidental. We report on a case of a 34-year-old male diagnosed with adult onset Still's disease and successfully treated with intravenous glucocorticoids. Nine months after the onset of adult Still’ s disease, the patient exhibited presence of asymptomatic thrombocytopenia during chloroquine and low dose glucocorticoid therapy. Physical status was unremarkable, except for pallor of the skin and mucosa. Laboratory evaluation revealed profound thrombocytopenia and hemolytic anemia. Coombs’ tests were negative. The renal function tests were all normal. Peripheral blood smear showed frequent schistocytes. Based on the presence of thrombocytopenia and microangiopathic hemolytic anemia, with the exclusion of other known causes, the patient was diagnosed with thrombotic microangiopathy and successfully treated with plasma exchange and intravenous glucocorticoids. Also, we review the literature concerning the association between adult onset Still's disease and thrombotic microangiopathy. This case is the fifteenth report in the literature on thrombotic microangiopathy associated with adult onset Still's disease. The mean age of adult Still's disease onset in these cases was 31.60 years. The interval between onset of thrombotic microangiopathy and the diagnosis of adult onset Still's disease ranged from 3 days to 17 years, and the female/male ratio was 2/1. In more than half of patients thrombotic microangiopathy occured within the first 6 months of establishing adult onset Still's disease. Eleven of fifteen patients (73%) were treated with plasmapheresis in addition to glucocorticoid therapy, and among them 8 (73%) had complete remission, whereas the other 3 patients had permanent visual impairment and/or digital ischemia. Among four patients who were not treated with plasmapheresis, two died, one developed end-stage renal disease, and one had complete remission. Awareness of the possible development of thrombotic microangiopathy in patients with adult Still's disease is critical, so as to initiate early treatment and to prevent complications and recurrence of thrombotic microangiopathy. Patients with adult onset Still’ s disease should be closely monitored for signs and symptoms of thrombotic microangiopathy during the first six months of establishing adult onset Still's disease.

Thrombotic microangiopathy ; adult onset Still's disease ; chloroquine phosphate ; thrombotic thrombocytopenic purpura ; hemolytic uremic syndrome

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