Severe systemic inflammatory response syndrome with shock and ARDS resulting from Still's disease - Clinical response with high-dose pulse methylprednisolone therapy

Adult-onset Still's disease, the adult variant of the systemic form of juve nile arthritis, is an uncommon systemic inflammatory disorder of unknown et iology characterized by high spiking fevers, neutrophilic leukocytosis, art hritis, and an evanescent rash. There is often a delay in reaching a firm d iagnosis. Differential diagnoses include infection, malignancy, and various immunologic disorders. Increased ferritin levels are of particular value i n establishing the diagnosis. Clinical response to high-dose corticosteroid s may be dramatic. We report a case of a 29-year-old woman who had recently been investigated for fever of unknown origin, and who presented to our ho spital with high fever and hypotension. Her condition rapidly deteriorated with the development of ARDS, disseminated intravascular coagulation, and s hock. The patient had a markedly elevated serum ferritin concentration of 2 6,000 ng/mL. High-dose pulse methylprednisolone therapy resulted in a remar kable clinical improvement. Such a severe case of systemic inflammatory res ponse syndrome, masquerading as septic shock, has not been reported previou sly.