Adult Onset Still's Disease Misunderstood as Sepsis with Bile Peritonitis

ABSTRACT

Adult onset Still's disease (AOSD) is a rare systemic inflammatory disorder of an unknown etiology, and its major clinical manifestations include high spiking fever, polyarthralgia, salmon-colored evanescent rash and neutrophilic leukocytosis. We describe here a 41 year old woman with AOSD who presented with non-remitting high fever, polyarthralgia, sore throat, skin rash, splenomegaly, thrombocytopenia, neutrophilic leukocytosis, hyperferritinemia and coagulopathy with disseminated intravascular coagulation (DIC). The patient had a history of laparoscopic cholecystectomy due to acalculous cholecystitis prior to admission. We suspected sepsis due to bile peritonitis after the previous laparoscopic cholecystectomy. Yet we could not detect infectious organisms on the cultures or serologic studies. Finally, we suspected AOSD-associated hemophagocytic syndrome (HS). So, intravenous immunoglobulin and pulse methylprednisolone treatment brought about transient improvement of the fever and the neutrophilic leukocytosis, but the disease progressed and the patient expired due to acute renal failure. HS is a fatal cause of AOSD. If a patient has DIC and sepsis and these fail to respond to conservative treatment, then AOSD should be added to the differential diagnosis of sepsis and DIC.