The diagnostic challenge of Adult-onset Still’s disease

Abstract

Adult-onset Still’s disease (AOSD) is a rare systemic inflammatory disease of unknown etiology, characterized by symptoms including high fever, salmon-pink rash, and also inflammatory symptoms such as sore throat, arthritis or arthralgia and polyserositis [1,2].The prevalence is around 1.5 cases per 100,000 - 1000,000 population. The age distribution of Still’s disease has two peaks, the first one at the age of 15 - 25 years and the second one at 35 - 45 years [3]. The most sensitive diagnostic criteria is the Yamaguchi criteria [3].At least 5 criteria are required for diagnosis, which should include at least 2 major ones. Elevated serum ferritin level is not yet considered as a criteria for diagnosis of AOSD in Yamaguchi criteria, however, there are several studies which have demonstrated a strong association between extremely high serum ferritin and AOSD [5]. This is a 44-year-old lady, who was admitted with the chief complaint of chills and fever since two weeks before admission. She complained of body pain, arthralgia, dry cough, sore throat and generalized maculopapular rash.she had a serum ferritin level of 9291 ng/ml. she was labeled AOSD.

Keywords: Adult Onset Still’s Disease; Fever of Unknown Origin, serum ferritin