Skip to main content

Adult-Onset Still’s Disease

  • Chapter
Primer on the Rheumatic Diseases
  • 104k Accesses

Abstract

The clinical features of adult Still’s disease resemble the systemic form of juvenile rheumatoid arthritis. The disorder is rare, affects both genders equally, and exists worldwide. The majority of patients are 16 to 35 years of age (1).

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

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 99.00
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 129.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

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

    Article  CAS  PubMed  Google Scholar 

  2. Pouchot J, Esdaile JM, Sampalis J, et al. Adult Still’s disease: manifestations, disease course, and outcome in 62 patients. Medicine 1991;70:118–136.

    Article  CAS  PubMed  Google Scholar 

  3. Sampalis JS, Medsger TA Jr, Fries JF, et al. Risk factors for adult Still’s disease. J Rheumatol 1996;23:2049–2054.

    CAS  PubMed  Google Scholar 

  4. Chen DY, Lan JL, Lin FJ, Hsieh TY, Wen MC. Predominance of Th1 cytokine in peripheral blood and pathological tissues of patients with active untreated adult onset Still’s disease. Ann Rheum Dis 2004;63:1300–1306.

    Article  CAS  PubMed  Google Scholar 

  5. Hoshino T, Ohta A, Yang D, et al. Elevated serum interleukin 6, interferon-gamma, and tumor necrosis factor-alpha levels in patients with adult Still’s disease. J Rheumatol 1998;25:396–398.

    CAS  PubMed  Google Scholar 

  6. Kawashima M, Yamamura M, Taniai M, et al. Levels of interleukin-18 and its binding inhibitors in the blood circulation of patients with adult-onset Still’s disease. Arthritis Rheum 2001;44:550–560.

    Article  CAS  PubMed  Google Scholar 

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

    CAS  PubMed  Google Scholar 

  8. Cush JJ, Medsger TA Jr, Christy WC, Herbert D, Co–operstein LA. Adult-onset Still’s disease: clinical course and outcome. Arthritis Rheum 1987;30:186–194.

    Article  CAS  PubMed  Google Scholar 

  9. Esdaile JM, Tannenbaum H, Lough JO, Hawkins D. Hepatic abnormalities in adult Still’s disease. J Rheumatol 1979;6:673–679.

    CAS  PubMed  Google Scholar 

  10. Medsger TA Jr, Christy WC. Carpal arthritis with ankylosis in late onset Still’s disease. Arthritis Rheum 1976;19:232–242.

    Article  PubMed  Google Scholar 

  11. Bjorkengren AG, Pathria MN, Sartoris DJ, et al. Carpal alterations in adult-onset Still’s disease, juvenile chronic arthritis and adult-onset rheumatoid arthritis: a comparative study. Radiology 1987;165:545–548.

    CAS  PubMed  Google Scholar 

  12. Yamaguchi M, Ohta A, Tsunematsu T, et al. Preliminary criteria for classification of adult Still’s disease. J Rheumatol 1992;19:424–430.

    CAS  PubMed  Google Scholar 

  13. Masson C, Le Loet X, Liote F, et al. Comparative study of 6 types of criteria in adult Still’s disease. J Rheumatol 1996;23:495–497.

    CAS  PubMed  Google Scholar 

  14. Fautrel B, Le Moel G, Saint-Marcoux B, et al. Diagnostic value of ferritin and glycosylated ferritin in adult onset Still’s disease: J Rheumatol 2001;28:322–329.

    CAS  PubMed  Google Scholar 

  15. Lambotte O, Cacoub P, Costedoat N, et al. High ferritin and low glycosylated ferritin may also be a marker of excessive macrophage activation. J Rheumatol 2003;30:1027–1028.

    CAS  PubMed  Google Scholar 

  16. Sampalis JS, Esdaile JM, Medsger TA Jr, et al. A controlled study of the long-term prognosis of adult Still’s disease. Am J Med 1995;98:384–388.

    Article  CAS  PubMed  Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

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

    CAS  PubMed  Google Scholar 

  19. Jung JH, Jun JB, Yoo DH, et al. High toxicity of sulfasalazine in adult-onset Still’s disease. Clin Exp Rheumatol 2000;18:245–248.

    CAS  PubMed  Google Scholar 

  20. Marchesoni A, Ceravolo GP, Battafarano N, Rossetti A, Tosi S, Fantini F. Cyclosporin A in the treatment of adult onset Still’s disease. J Rheumatol 1997;24:1582–1587.

    CAS  PubMed  Google Scholar 

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

    CAS  PubMed  Google Scholar 

  22. Bennett AN, Peterson P, Sangle S, et al. Adult onset Still’s disease and collapsing glomerulopathy: successful treatment with intravenous immunoglobulins and mycophenolate mofetil. Rheumatology 2004;43:795–799.

    Article  CAS  PubMed  Google Scholar 

  23. Kokkinos A, Iliopoulos A, Greka P, Efthymiou A, Katsilambros N, Sfikakis PP. Successful treatment of refractory adult-onset Still’s disease with infliximab. A prospective, non-comparative series of four patients. Clin Rheumatol 2004;23:45–49.

    Article  PubMed  Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

  26. Fitzgerald AA, LeClercq SA, Yan A, Homik JE, Dinarello CA. Rapid response to anakinra in patients with refractory adults Still’s disease. Arthritis Rheum 2005;52:1794–1803.

    Article  CAS  PubMed  Google Scholar 

  27. Vasques Godinho FM, Parreira Santos MJ, Canas da Silva J. Refractory adult onset Still’s disease successfully treated with anakinra. Ann Rheum Dis 2005;64:647–648.

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2008 Springer Science+Business Media, LLC

About this chapter

Cite this chapter

Esdaile, J.M. (2008). Adult-Onset Still’s Disease. In: Klippel, J.H., Stone, J.H., Crofford, L.J., White, P.H. (eds) Primer on the Rheumatic Diseases. Springer, New York, NY. https://doi.org/10.1007/978-0-387-68566-3_23

Download citation

  • DOI: https://doi.org/10.1007/978-0-387-68566-3_23

  • Publisher Name: Springer, New York, NY

  • Print ISBN: 978-0-387-35664-8

  • Online ISBN: 978-0-387-68566-3

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics