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 juvenile arthritis, is an uncommon systemic inflammatory disorder of unknown etiology characterized by high spiking fevers, neutrophilic leukocytosis, arthritis, and an evanescent rash. There is often a delay in reaching a firm diagnosis. Differential diagnoses include infection, malignancy, and various immunologic disorders. Increased ferritin levels are of particular value in establishing the diagnosis. Clinical response to high-dose corticosteroids 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 hospital with high fever and hypotension. Her condition rapidly deteriorated with the development of ARDS, disseminated intravascular coagulation, and shock. The patient had a markedly elevated serum ferritin concentration of 26,000 ng/mL. High-dose pulse methylprednisolone therapy resulted in a remarkable clinical improvement. Such a severe case of systemic inflammatory response syndrome, masquerading as septic shock, has not been reported previously.