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Therapeutic responses and prognosis in adult-onset Still’s disease

Rheumatology International volume 32, pages 1291–1298 (2012)Cite this article

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.

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Acknowledgments

This study was supported by the Brain Korea 21 project for Ajou University School of Medicine, Ministry of Health & Welfare, R.O.K. (A050571).

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We have no conflict of interest.

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Affiliations

  1. 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

    Hyoun-Ah Kim, Jun-Mo Sung & Chang-Hee Suh

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  1. Hyoun-Ah Kim
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  2. Jun-Mo Sung
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  3. Chang-Hee Suh
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Correspondence to Chang-Hee Suh.

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Kim, HA., Sung, JM. & Suh, CH. Therapeutic responses and prognosis in adult-onset Still’s disease. Rheumatol Int 32, 1291–1298 (2012). https://doi.org/10.1007/s00296-011-1801-6

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  • DOI: https://doi.org/10.1007/s00296-011-1801-6

Keywords

  • Adult-onset Still’s disease
  • Treatment
  • Prognosis
  • Corticosteroids
  • Intravenous immunoglobulin
  • Methotrexate