title: Therapeutic responses and prognosis in adult-onset Still's disease.
abstract: To date, the treatment of adult-onset Still's disease (AOSD) has been largely empirical; therefore, this study was conducted to investigate the response to therapy and prognostic factors of AOSD. Fifty-four Korean patients with AOSD were enrolled based on Yamaguchi's criteria. We retrospectively analyzed the treatments and prognosis. Thirty-nine patients (72.2%) were female, and the average age at disease onset was 37.3 years. Twenty-nine patients had a monocyclic disease (53.7%), five had a polycyclic (9.3%) and fifteen had a chronic articular disease (27.7%) and five died (9.3%). The elevated ESR and corticosteroids refractoriness were associated with poor prognosis (P = 0.023 and P = 0.009, respectively). The patients that died were older than those survived (49.2 ± 11.8 vs. 42.2 ± 14 year old, P = 0.024). Forty-two patients were treated with non-steroidal anti-inflammatory drugs; however, they also needed corticosteroids and intravenous immunoglobulin (IVIG). Among 50 patients treated with high-dose corticosteroids, 21 patients (42%) were resistant to corticosteroids and treated with IVIG or anti-tumor necrosis factor (TNF) agents. Of the 23 patients medicated with IVIG, the prognosis was better in IVIG-responsive patients, indicating a therapeutic effect. Methotrexate was the most commonly used disease modifying anti-rheumatic drugs (27 patients, 50%), and the corticosteroid requirements were lower in the methotrexate-responsive patients. Approximately half of AOSD patients had a poor prognosis and were corticosteroids resistance. An elevated ESR and non-response to corticosteroids were associated with poor prognosis. Patients who died were older than those survived.
authors.affiliation: Department of Allergy and Rheumatology, and BK21 Division of Cell Transformation and Restoration, Ajou University School of Medicine, Woncheon-dong San 5, Youngtong-gu, Suwon 443-721, Korea.
authors.forename: Hyoun-Ah
authors.initials: HA
authors.lastname: Kim
authors.forename: Jun-Mo
authors.initials: JM
authors.lastname: Sung
authors.forename: Chang-Hee
authors.initials: CH
authors.lastname: Suh
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use*; D000328:Adult; D000367:Age Factors; D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000627:therapeutic use*; D001799:Blood Sedimentation; D016009:Chi-Square Distribution; D004351:Drug Resistance; D004359:Drug Therapy, Combination; D005260:Female; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use*; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D011237:Predictive Value of Tests; D056910:Republic of Korea; D012189:Retrospective Studies; D018570:Risk Assessment; D012307:Risk Factors; D016706:Still's Disease, Adult-Onset / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy* / Q000401:mortality; D016019:Survival Analysis; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D000305:Adrenal Cortex Hormones; D000894:Anti-Inflammatory Agents, Non-Steroidal; D016756:Immunoglobulins, Intravenous; D007166:Immunosuppressive Agents