Adult-Onset Still’s Disease

  • John M. Esdaile


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).


Mycophenolate Mofetil Juvenile Rheumatoid Arthritis Salmon Pink Elevated White Blood Cell Count Intravascular Coagulopathy 


  1. 1.
    Efthimiou P, Paik PK, Bielory L. Diagnosis and management of adult onset Still’s disease. Ann Rheum Dis 2006;65:564–572.CrossRefPubMedGoogle Scholar
  2. 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.CrossRefPubMedGoogle Scholar
  3. 3.
    Sampalis JS, Medsger TA Jr, Fries JF, et al. Risk factors for adult Still’s disease. J Rheumatol 1996;23:2049–2054.PubMedGoogle Scholar
  4. 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.CrossRefPubMedGoogle Scholar
  5. 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.PubMedGoogle Scholar
  6. 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.CrossRefPubMedGoogle Scholar
  7. 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.PubMedGoogle Scholar
  8. 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.CrossRefPubMedGoogle Scholar
  9. 9.
    Esdaile JM, Tannenbaum H, Lough JO, Hawkins D. Hepatic abnormalities in adult Still’s disease. J Rheumatol 1979;6:673–679.PubMedGoogle Scholar
  10. 10.
    Medsger TA Jr, Christy WC. Carpal arthritis with ankylosis in late onset Still’s disease. Arthritis Rheum 1976;19:232–242.CrossRefPubMedGoogle Scholar
  11. 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.PubMedGoogle Scholar
  12. 12.
    Yamaguchi M, Ohta A, Tsunematsu T, et al. Preliminary criteria for classification of adult Still’s disease. J Rheumatol 1992;19:424–430.PubMedGoogle Scholar
  13. 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.PubMedGoogle Scholar
  14. 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.PubMedGoogle Scholar
  15. 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.PubMedGoogle Scholar
  16. 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.CrossRefPubMedGoogle Scholar
  17. 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.CrossRefPubMedGoogle Scholar
  18. 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.PubMedGoogle Scholar
  19. 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.PubMedGoogle Scholar
  20. 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.PubMedGoogle Scholar
  21. 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.PubMedGoogle Scholar
  22. 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.CrossRefPubMedGoogle Scholar
  23. 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.CrossRefPubMedGoogle Scholar
  24. 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.CrossRefPubMedGoogle Scholar
  25. 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.CrossRefPubMedGoogle Scholar
  26. 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.CrossRefPubMedGoogle Scholar
  27. 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.CrossRefPubMedGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2008

Authors and Affiliations

  • John M. Esdaile
    • 1
    • 2
  1. 1.Arthritis Research Centre of CanadaCanada
  2. 2.University of British ColumbiaVancouverCanada

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