ORIGINAL ARTICLE Adult-onset Still’s disease: a report of 28 cases and review of the literature Golbarg Mehrpoor Æ Mohammad Bagher Owlia Æ Hossein Soleimani Æ Jamshid Ayatollahi Received: 7 January 2008 / Accepted: 16 April 2008 / Published online: 28 August 2008 Ó Japan College of Rheumatology 2008 Abstract Adult-onset Still’s disease (AOSD) is a rare systemic inflammatory disorder of unknown etiology. It is characterized by fever, skin rash, polyarthralgias or polyar- thritis, sore throat, hepatosplenomegaly, lymphadenopathy, leukocytosis, liver enzyme elevation, and high serum level of ferritin. Several kinds of skin lesions have been reported in this condition. The aim of this study was to assess the clinical and laboratory aspects of 28 patients with AOSD in central Iran. According to the diagnostic criteria of AOSD, we identified 28 patients between 2002 and 2007. We intended to describe the clinical characteristics, treatment, and out- come of our patients with AOSD. Of 28 patients with AOSD, 21 (75%) were female, 7 (25%) were male. Fever (100%), sore throat (92%), Arthralgia (92%), dermatographism (92%), typical rash (85%) and arthritis (60%) were the most common findings. The mean values of laboratory findings were as follows; C-reactive protein (CRP) level of 14.4 mg/dl, erythrocyte sedimentation rate (ESR) of 91.5 mm/h, leukocyte count of 15744.4/ll. Abnormal levels of aspartate aminotransferase and alanine aminotransferase were observed in 25 (89%) patients. Twenty patients (71%) had high ferritin values ( [ 500 ng/ml). The clinical characteris- tics were similar to previous series. A febrile polyarthritis was the most frequent presentation form. Dermatographism was frequently encountered phenomenon in our patients with AOSD. Being that dermatographism is a simple inducible skin reaction, along with its sensitivity in active disease, we suggest more controlled studies to validate accuracy and positive predictive value of it in convenient clinical setting in the diagnosis of AOSD and to consider including it in diagnostic criteria. Keywords Adult-onset Still’s disease (AOSD) Á Dermatographism Á FUO Á Rheumatoid arthritis Á Dermographism Introduction The term adult-onset Still’s disease (AOSD) was used to describe patients who had not fulfilled criteria for classic rheumatoid arthritis but had signs and symptoms similar to the children with systemic onset juvenile rheumatoid arthritis [1]. It is a rare systemic disorder of unknown eti- ology characterized by spiking fever, evanescent rash, arthritis and multi-organ involvement. The first description of an adult patient with signs and symptoms of AOSD, (erroneously labelled rheumatoid arthritis), was published in 1896. Then in 1897 it was described by George Still in 22 patients [2]. In 1971, Eric Bywaters described 14 adults with similar presentation, and termed it adult-onset Still’s disease (AOSD) [1, 3, 4]. Because there is no pathogonomic test for the disease, diagnosis is based on a set of clinical and laboratory criteria G. Mehrpoor Department of Internal Medicine, Sadoughi Hospital, Shahid Sadoughi University of Medical Sciences, Safaeieh, Yazd, Iran e-mail: golbargmehrpoor@yahoo.com; golbargmehrpoor@gmail.com M. B. Owlia (&) Á H. Soleimani Department of Rheumatology, Shahid Sadoughi University of Medical Sciences, Yazd, Iran e-mail: owlia@ssu.ac.ir H. Soleimani e-mail: dhsol33@yahoo.com J. Ayatollahi Department of Infectious Diseases, Shahid Sadoughi University of Medical Sciences, Yazd, Iran e-mail: jamshidayatollahi@yahoo.com 123 Mod Rheumatol (2008) 18:480–485 DOI 10.1007/s10165-008-0104-6