Skip to main content

Advertisement

Log in

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

  • Original Article
  • Published:
Rheumatology International Aims and scope Submit manuscript

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.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1

Similar content being viewed by others

References

  1. Bywaters EG (1971) Still’s disease in the adult. Ann Rheum Dis 30:121–133

    Article  PubMed  CAS  Google Scholar 

  2. Fautrel B (2008) Adult-onset Still disease. Best Pract Res Clin Rheumatol 22:773–792

    Article  PubMed  Google Scholar 

  3. Ohta A, Yamaguchi M, Kaneoka H et al (1987) Adult Still’s disease: review of 228 cases from the literature. J Rheumatol 14:1139–1146

    PubMed  CAS  Google Scholar 

  4. Pouchot J, Sampalis JS, Beaudet F et al (1991) Adult Still’s disease: manifestations, disease course, and outcome in 62 patients. Medicine (Baltimore) 70:118–136

    CAS  Google Scholar 

  5. Cagatay Y, Gul A, Cagatay A et al (2009) Adult-onset Still’s disease. Int J Clin Pract 63:1050–1055

    Article  PubMed  CAS  Google Scholar 

  6. Efthimiou P, Kontzias A, Ward CM et al (2007) Adult-onset Still’s disease: can recent advances in our understanding of its pathogenesis lead to targeted therapy? Nat Clin Pract Rheumatol 3:328–335

    Article  PubMed  CAS  Google Scholar 

  7. Magadur-Joly G, Billaud E, Barrier JH et al (1995) Epidemiology of adult Still’s disease: estimate of the incidence by a retrospective study in west France. Ann Rheum Dis 54:587–590

    Article  PubMed  CAS  Google Scholar 

  8. Wakai K, Ohta A, Tamakoshi A et al (1997) Estimated prevalence and incidence of adult Still’s disease: findings by a nationwide epidemiological survey in Japan. J Epidemiol 7:221–225

    Article  PubMed  CAS  Google Scholar 

  9. Coffernils M, Soupart A, Pradier O et al (1992) Hyperferritinemia in adult onset Still’s disease and the hemophagocytic syndrome. J Rheumatol 19:1425–1427

    PubMed  CAS  Google Scholar 

  10. Reginato AJ, Schumacher HR Jr, Baker DG et al (1987) Adult onset Still’s disease: experience in 23 patients and literature review with emphasis on organ failure. Semin Arthritis Rheum 17:39–57

    Article  PubMed  CAS  Google Scholar 

  11. Hirohata S, Kamoshita H, Taketani T et al (1986) Adult Still’s disease complicated with adult respiratory distress. Arch Intern Med 146:2409–2410

    Article  PubMed  CAS  Google Scholar 

  12. Akritidis N, Sakkas LI (1995) Adult onset Still’s disease. 16 cases. Presse Med 24:1207–1208

    PubMed  CAS  Google Scholar 

  13. Andres E, Kurtz JE, Perrin AE et al (2003) Retrospective monocentric study of 17 patients with adult Still’s disease, with special focus on liver abnormalities. Hepatogastroenterology 50:192–195

    PubMed  Google Scholar 

  14. Evensen KJ, Nossent HC (2006) Epidemiology and outcome of adult-onset Still’s disease in Northern Norway. Scand J Rheumatol 35:48–51

    Article  PubMed  CAS  Google Scholar 

  15. Chen DY, Lan JL, Hsieh TY et al (2004) Clinical manifestations, disease course, and complications of adult-onset Still’s disease in Taiwan. J Formos Med Assoc 103:844–852

    PubMed  Google Scholar 

  16. Ohta A, Yamaguchi M, Tsunematsu T et al (1990) Adult Still’s disease: a multicenter survey of Japanese patients. J Rheumatol 17:1058–1063

    PubMed  CAS  Google Scholar 

  17. Uppal SS, Pande IR, Kumar A et al (1995) Adult onset Still’s disease in northern India: comparison with juvenile onset Still’s disease. Br J Rheumatol 34:429–434

    Article  PubMed  CAS  Google Scholar 

  18. Cush JJ, Medsger TA Jr, Christy WC et al (1987) Adult-onset Still’s disease. Clinical course and outcome. Arthritis Rheum 30:186–194

    Article  PubMed  CAS  Google Scholar 

  19. Tan EM, Cohen AS, Fries JF et al (1982) The 1982 revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 25:1271–1277

    Article  PubMed  CAS  Google Scholar 

  20. Pay S, Turkcapar N, Kalyoncu M et al (2006) A multicenter study of patients with adult-onset Still’s disease compared with systemic juvenile idiopathic arthritis. Clin Rheumatol 25:639–644

    Article  PubMed  Google Scholar 

  21. Zeng T, Zou YQ, Wu MF et al (2009) Clinical features and prognosis of adult-onset still’s disease: 61 cases from China. J Rheumatol 36:1026–1031

    Article  PubMed  CAS  Google Scholar 

  22. Fitzgerald AA, Leclercq SA, Yan A et al (2005) Rapid responses to anakinra in patients with refractory adult-onset Still’s disease. Arthritis Rheum 52:1794–1803

    Article  PubMed  CAS  Google Scholar 

  23. Fautrel B, Sibilia J, Mariette X et al (2005) Tumour necrosis factor alpha blocking agents in refractory adult Still’s disease: an observational study of 20 cases. Ann Rheum Dis 64:262–266

    Article  PubMed  CAS  Google Scholar 

  24. Kokkinos A, Iliopoulos A, Greka P et al (2004) Successful treatment of refractory adult-onset Still’s disease with infliximab. A prospective, non-comparative series of four patients. Clin Rheumatol 23:45–49

    Article  PubMed  Google Scholar 

  25. Benucci M, Li GF, Del Rosso A et al (2005) Adalimumab (anti-TNF-alpha) therapy to improve the clinical course of adult-onset Still’s disease: the first case report. Clin Exp Rheumatol 23:733

    PubMed  CAS  Google Scholar 

  26. Efthimiou P, Georgy S (2006) Pathogenesis and management of adult-onset Still’s disease. Semin Arthritis Rheum 36:144–152

    Article  PubMed  CAS  Google Scholar 

  27. Maier J, Birkenfeld G, Pfirstinger J et al (2008) Effective treatment of steroid refractory adult-onset Still’s disease with anakinra. J Rheumatol 35:939–941

    PubMed  CAS  Google Scholar 

  28. Lequerre T, Quartier P, Rosellini D et al (2008) Interleukin-1 receptor antagonist (anakinra) treatment in patients with systemic-onset juvenile idiopathic arthritis or adult onset Still disease: preliminary experience in France. Ann Rheum Dis 67:302–308

    Article  PubMed  CAS  Google Scholar 

  29. Naumann L, Feist E, Natusch A et al (2010) IL1-receptor antagonist anakinra provides long-lasting efficacy in the treatment of refractory adult-onset Still’s disease. Ann Rheum Dis 69:466–467

    Article  PubMed  CAS  Google Scholar 

  30. Kotter I, Wacker A, Koch S et al (2007) Anakinra in patients with treatment-resistant adult-onset Still’s disease: four case reports with serial cytokine measurements and a review of the literature. Semin Arthritis Rheum 37:189–197

    Article  PubMed  Google Scholar 

  31. Mahmud T, Hughes GR (1999) Intravenous immunoglobulin in the treatment of refractory adult Still’s disease. J Rheumatol 26:2067–2068

    PubMed  CAS  Google Scholar 

  32. Permal S, Wechsler B, Cabane J et al (1995) Treatment of Still disease in adults with intravenous immunoglobulins. Rev Med Interne 16:250–254

    Article  PubMed  CAS  Google Scholar 

  33. Vignes S, Wechsler B, Amoura Z et al (1998) Intravenous immunoglobulin in adult Still’s disease refractory to non-steroidal anti-inflammatory drugs. Clin Exp Rheumatol 16:295–298

    PubMed  CAS  Google Scholar 

  34. Efthimiou P, Paik PK, Bielory L (2006) Diagnosis and management of adult onset Still’s disease. Ann Rheum Dis 65:564–572

    Article  PubMed  CAS  Google Scholar 

  35. Kraetsch HG, Antoni C, Kalden JR et al (2001) Successful treatment of a small cohort of patients with adult onset of Still’s disease with infliximab: first experiences. Ann Rheum Dis 60:55–57

    Article  Google Scholar 

  36. Husni ME, Maier AL, Mease PJ et al (2002) Etanercept in the treatment of adult patients with Still’s disease. Arthritis Rheum 46:1171–1176

    Article  PubMed  CAS  Google Scholar 

  37. Fautrel B, Borget C, Rozenberg S et al (1999) Corticosteroid sparing effect of low dose methotrexate treatment in adult Still’s disease. J Rheumatol 26:373–378

    PubMed  CAS  Google Scholar 

  38. Fujii T, Akizuki M, Kameda H et al (1997) Methotrexate treatment in patients with adult onset Still’s disease–retrospective study of 13 Japanese cases. Ann Rheum Dis 56:144–148

    Article  PubMed  CAS  Google Scholar 

  39. Kontzias A, Efthimiou P (2008) Adult-onset Still’s disease: pathogenesis, clinical manifestations and therapeutic advances. Drugs 68:319–337

    Article  PubMed  CAS  Google Scholar 

Download references

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).

Conflict of interest

We have no conflict of interest.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Chang-Hee Suh.

Rights and permissions

Reprints and permissions

About this article

Cite this article

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

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

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

Keywords

Navigation