Inadvertent Skipping of Steroids in Septic Shock Leads to a Diagnosis of Adult Onset Still’s Disease

Abstract

Adult onset Still’s disease is uncommon in middle-aged and elderly individuals and can rarely present with shock; shock is usually associated with disseminated intravascular coagulation, multiorgan dysfunction syndrome or acute respiratory distress syndrome. We report a post-menopausal woman with arthritis, fever, pneumonitis and hypotension which was managed as septic shock. Steroids were inadvertently missed during the second day of hospitalization in the intensive care unit. Persistence of hypotension on inotropes, with normal renal, hepatic and neurological function and recurrence of fever when steroids were skipped, led to suspicion of an inflammatory disorder. A diagnosis of Still’s disease may be entertained in postmenopausal women with polyarthritis, rash, and fever with leukocytosis. Sepsis is mimicked, and multiple antibiotics use is common before the diagnosis of such an entity is made. Shock is rare in adult onset Still’s disease and is not necessarily associated with disseminated intravascular coagulation, acute respiratory distress syndrome, or multiorgan dysfunction.

Adult Onset Still’s Disease - a rare diagnosis of undetermined febrile syndrome

“…As for arthritis the predominantly affected joints are the knees, wrists, ankles and hands. 1 The rash is generally coincident with febrile peaks, manifesting itself in the trunk and proximal extremities. 1 The typical manifestations of AOSD are arthralgias, evanescent cutaneous rash, spiking fever and odynophagia.…”

“…1 The rash is generally coincident with febrile peaks, manifesting itself in the trunk and proximal extremities. 1 The typical manifestations of AOSD are arthralgias, evanescent cutaneous rash, spiking fever and odynophagia. Other signs and symptoms, such as myalgias, weight loss, lymphadenopathy, hepatosplenomegaly, pleuritis, myocarditis, and pericarditis are also described.…”