UpToDate performs a continuous review of over 350 journals and other resources. Updates are added as important new information is published. The literature review for version 14.1 is current through December 2005; this topic was last changed on May 13, 2005. The next version of UpToDate (14.2) will be released in June 2006.

INTRODUCTION — Adult Still's disease (ASD) is an inflammatory disorder characterized by daily, spiking high fevers, arthritis, and an evanescent rash. First described in children by George Still in 1896, "Still's disease" became the eponymous term for juvenile rheumatoid arthritis and is now called systemic onset juvenile rheumatoid arthritis [1]. (See "Clinical manifestation and diagnosis of systemic onset juvenile rheumatoid arthritis"). In 1971, ASD was used to describe a series of adult patients who did not fulfill criteria for classic rheumatoid arthritis but had features similar to the children with systemic onset juvenile rheumatoid arthritis [2].

ETIOLOGY — The etiology of ASD is unknown. A variety of infectious triggers have been suggested, including the following viruses: rubella, echovirus 7, mumps, Epstein-Barr, cytomegalovirus, parainfluenza, and parvovirus [3-6]. Suspected bacterial pathogens include Yersinia enterocolitica and Mycoplasma pneumoniae [5,7].
It has also been suggested that genetic factors may be important. In one series of 62 French patients, for example, HLA-B17, B18, B35, and DR2 were significantly associated with ASD [8]. However, other studies have not confirmed these findings [9]. Familial cases are very uncommon and there have been few reports of cases in twins [10].

EPIDEMIOLOGY — A retrospective French study estimated the incidence of ASD to be 0.16 cases per 100,000 persons per year with an equal sex distribution [11]. There is a bimodal age distribution, with one peak between the ages of 15 and 25 and the second between the ages of 36 and 46. However, occasional patients present with ASD after the age of 70 [12,13].

CLASSIFICATION CRITERIA — There is no specific test or combination of tests that can be used to establish the diagnosis of ASD. Thus, a number of different classification criteria have been proposed for the purpose of classification. To establish a diagnosis of ASD requires the presence of certain major or minor criteria or a combination of both and the absence of ce [continua..]