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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.
AuthorsHyoun-Ah Kim, Jun-Mo Sung, Chang-Hee Suh
JournalRheumatology international (Rheumatol Int) Vol. 32 Issue 5 Pg. 1291-8 (May 2012) ISSN: 1437-160X [Electronic] Germany
PMID21274538 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
Chemical References
  • Adrenal Cortex Hormones
  • Anti-Inflammatory Agents, Non-Steroidal
  • Immunoglobulins, Intravenous
  • Immunosuppressive Agents
Topics
  • Adrenal Cortex Hormones (therapeutic use)
  • Adult
  • Age Factors
  • Anti-Inflammatory Agents, Non-Steroidal (therapeutic use)
  • Blood Sedimentation
  • Chi-Square Distribution
  • Drug Resistance
  • Drug Therapy, Combination
  • Female
  • Humans
  • Immunoglobulins, Intravenous (therapeutic use)
  • Immunosuppressive Agents (therapeutic use)
  • Male
  • Middle Aged
  • Predictive Value of Tests
  • Republic of Korea
  • Retrospective Studies
  • Risk Assessment
  • Risk Factors
  • Still's Disease, Adult-Onset (complications, diagnosis, drug therapy, mortality)
  • Survival Analysis
  • Time Factors
  • Treatment Outcome

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