Hemolytic uremic syndrome/thrombotic thrombocytopenic purpura (HUS/TTP) complicating adult Still's disease: remission induced with intravenous immunoglobulin G
Coexistence of hemolytic uremic syndrome/thrombotic thrombocytopenic purpura (HUS/TTP) and adult Still's disease is extremely rare. We describe the case history of a 22-year-old young man who presented with evidence of a thrombotic microangiopathy complicated by dialysis-dependent renal failure, encephalopathy, and an ischemic retinopathy. The most important and novel feature of this case was the dramatic and sustained clinical remission of the TMA induced by intravenous immunoglobulin (IVIg) after failure of plasmapheresis and glucocorticoids to do so.