A multicenter study of patients with adult-onset Still’s disease compared with systemic juvenile idiopathic arthritis

Abstract

Adult-onset Still's disease (AOSD) has often been regarded as the adult spectrum of systemic juvenile idiopathic arthritis (sJIA). The present study aims to compare the clinical and laboratory features, the disease course and the response to treatment in patients having AOSD with those having sJIA. Retrospective review of all available data that were filled out by adult and paediatric rheumatologists from six centers using a standard data extraction form was performed. A total of 95 patients with AOSD and 25 patients with sJIA were recruited for the study. The frequency of fever, rash, myalgia, weight loss and sore throat was higher in patients with AOSD. The pattern of joint involvement differed slightly. Laboratory findings were similar in both groups, except that liver dysfunction and neutrophilia were more common among adults. A multiphasic pattern dominated the childhood cases, whereas the most frequent course was a chronic one in adults. Corticosteroids and methotrexate were the most commonly employed therapy; however, chloroquine was another popular therapy in the adult group. We showed a difference in the rate of clinical and laboratory features between patients with AOSD and those with sJIA. AOSD and sJIA may still be the same disease, and children may simply be reacting differently as the result of the first encounter of the putative antigens with the immune system.

Cytokine profile in adult-onset Still's disease: Comparison with systemic juvenile idiopathic arthritis

“…In addition to clinical symptoms, most laboratory abnormalities were common to both patients with s-JIA and those with AOSD. However, serum ferritin levels were significantly higher in patients with AOSD than in those with s-JIA [5,6]. These findings were comparable with those of previous studies.…”

“…In support of this view, the present study found no differences between these conditions except for hepatomegaly and splenomegaly, which were more frequently observed in AOSD, and arthritis, which was more frequently observed in s-JIA. Previous studies reported significant differences between s-JIA and AOSD with regard to the frequencies of fever, skin rash, and sore throat, all of which occurred more frequently with AOSD [3,6]. The enrollment of more pediatric patients in our study relative to previous studies on clinical disease expression might account for the observed discrepancies.…”

“…Previous studies that compared the clinical features and laboratory findings of s-JIA and AOSD revealed that these two entities share many common characteristics and might actually represent the same clinical spectrum, thus differing only in age distribution [3][4][5][6]. In support of this view, the present study found no differences between these conditions except for hepatomegaly and splenomegaly, which were more frequently observed in AOSD, and arthritis, which was more frequently observed in s-JIA.…”

“…A comparison of the epidemiologies, genetic backgrounds, clinical presentations, and courses of these diseases convincingly suggests that s-JIA and AOSD are identical [3][4][5]. However, some differences between these disorders have been reported, including an increased frequency of fever, skin rash, myalgia, sore throat, weight loss, liver dysfunction, and neutrophilia in AOSD relative to s-JIA [3,6]. The precise AOSD pathogenesis remains to be elucidated, and it remains unknown whether s-JIA and AOSD share the same pathogenesis.…”

Adult - onsetStill′s disease: A review

“…It occurs worldwide and has been reported from France, [5,6] Norway, [7] Japan, [8,9] Greece, [10] USA, [11] Taiwan, [12,13] Spain, [14] Korea, [15,16] China, [17,18] Turkey, [19,20] India [21,22] and Iran. [23] The disease affects young people and has a bimodal age distribution with two peaks -at 15-25 and 36-46 years of age.…”