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Adult-Onset Still Disease Masqueraded as Sepsis

Diaz Zamora, Giancarlo MD*; Aguilar, Javier MD; Brar, Indira MD

Infectious Diseases in Clinical Practice: September 2010 - Volume 18 - Issue 5 - p 341-343
doi: 10.1097/IPC.0b013e3181d6516b
Case Reports

Adult-onset Still disease (AOSD) is a rare systemic inflammatory disease, associated with characteristic clinical and laboratory findings, which, if interpreted incorrectly, may lead to errors in the diagnosis, delay treatment, and produce significant morbidity and mortality. We present a case illustrating how AOSD may masquerade as sepsis. Our patient is a 59-year-old woman admitted with high fever, severe arthralgias, myalgias, sore throat, evanescent maculopapular rash, leukocytosis, and elevated ferritin. On initial evaluation, sepsis was the first consideration; however, despite broad-spectrum antibiotics, the patient did not improve clinically. Finally, the diagnosis of AOSD was made based on Yamaguchi's criteria. Systemic corticosteroid treatment was started along with discontinuation of antibiotic therapy, with dramatic improvement after 3 days. Adult-onset Still disease can mimic sepsis and needs to be suspected if there is no response to antibiotics in the setting of an evanescent rash that tends to occur with fever. A discussion based on a review of the literature is presented.

From the *Department of Internal Medicine and †Division of Infectious Diseases, Henry Ford Hospital, Detroit, MI.

Correspondence to: Giancarlo Diaz Zamora, MD, Department of Internal Medicine, Henry Ford Hospital, Detroit, MI 48202. E-mail:

The authors have no funding or conflicts of interest to disclose.

© 2010 Lippincott Williams & Wilkins, Inc.