Background:Adult-Onset Still’s disease (AOSD) is an autoinflammatory condition characterized by fever, rash, and arthritis. The diagnosis of AOSD is made by excluding common causes of fever of unknown origin which are infections, malignancies, autoimmune conditions and medication adverse effects. As it is a diagnostic challenge, further data on highlighting clinical and laboratory findings are necessary on guiding clinicians.Objectives:Our main objective is to present our single tertiary center experience of patients diagnosed with AOSD.Methods:This retrospective study was conducted at a tertiary rheumatology center. Patients were diagnosed with AOSD using Yamaguchi’s criteria and followed between 2007 and 2020. Demographic, clinical and laboratory information was retrieved from the patient chars. Treatment-related and prognostic information were also noted with additional information from phone call interviews.Results:The study includes 69 patients (23 M, 46 F). The mean age of diagnosis was 33.86±14.3. The presenting signs and symptoms of the patients are shown in Figure 1. The laboratory findings supporting the diagnosis at initial encounter are summarized in Table 1. The mean corticosteroid dose at initial diagnosis was 29.7±18 mg. In addition to corticosteroid treatment these patients were followed with different glucocorticoid-sparing agents. Methotrexate was the choice of treatment in 54 patients with the mean dose of 14.5±3.43 mg. Eight patients were treated with leflunomide, seven with anti-TNF agents, seven with tocilizumab, nineteen with anakinra and four with canakinumab.Figure 1.The presenting signs and symptoms of the patientsConclusion:In conclusion, the most common presenting symptoms in our AOSD cohort were fever and salmon-colored rash. In the differential diagnosis of fever of unknown origin especially with rash, AOSD should be considered. Corticosteroid was the main treatment modality. In patients who are unresponsive to conventional immunosuppressive treatment, biologic agents can be an alternative.Table 1.The laboratory findings at initial encounterMean ± Standard DeviationFerritin (ng/mL)3179.46±6503.56ESR (mm/h)77.43±28.47CRP (mg/L)102.29±70.39Leukocyte Count (cells/L)13147.3±4640.9ESR (mm/h)80±28.48CRP (mg/L)105.15±54.67Leukocyte Count (cells/L)12427.14±6530.43Disclosure of Interests:None declared
ADULT ONSET STILL'S DISEASE AS FEVER OF UNKNOWN ORIGIN - A CASE REPORT
