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Clinical features and prognosis in adult-onset still's disease: a study of 104 cases

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Abstract

The objective of this study is to evaluate the clinical features and prognosis of adult-onset Still's disease (AOSD). One hundred and four AOSD patients who were analyzed retrospectively were enrolled in this study. Medical charts were systematically reviewed for: demographic data, clinical features, laboratory findings, treatments, and outcomes. The major clinical features were: spiking fever 100%, evanescent maculopapular rash 95%, polyarthralgia 90%, sore throat 78%, lymphadenopathy 66%, hepatosplenomegaly 57%, hydrohymenitis 30%, neutrophilia 98%, liver disfunction 62%, increased erythrocyte sedimentation rate (ESR) 96%, and hyperferritinaemia 99%. Reactive hyperplasia was shown in all patients who underwent lymph node biopsy. Ninety-five percent and 63% of the patients were treated with glucocorticoid and immune suppressant, respectively. Those with prednisone or its equivalent dosage of ≥0.8 mg/kg/d achieved quicker remission and less relapse. Persistent fever, evanescent rash, arthritis, and sore throat were the most prevalent symptoms in patients with AOSD, with laboratory findings of leukocytosis, elevated liver enzymes, elevated ESR and serum ferritin. Glucocorticoid and immune suppressive drugs are effective for AOSD; however, the relapsing rate is relatively high. High levels of white blood cells, serum ferritin and ESR, as well as glucocorticoid dosage were related to relapse.

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References

  1. Singh S, Samant R, Joshi VR (2008) Adult onset Still's disease: a study of 14 cases. Clin Rheumatol 27(1):35–39

    Article  CAS  PubMed  Google Scholar 

  2. Meijvis SC, Endeman H, Geers AB et al (2007) Extremely high serum ferritin levels as diagnostic tool in adult-onset Still's disease. Neth J Med 65:212–214

    CAS  PubMed  Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

  4. Yamaguchi M, Ohta A, Tsunematsu T et al (1992) Preliminary criteria for classification of adult Still's disease. J Rheumatol 19:424–430

    CAS  PubMed  Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

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

    CAS  PubMed  Google Scholar 

  7. Appenzeller S, Castro GR, Costallat LT et al (2005) Adult-onset Still disease in southeast Brazi. J Clin Rheumatol 11:76–80

    Article  PubMed  Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

  9. Kádár J, Petrovicz E (2004) Adult-onset Still's disease. Best Pract Res Clin Rheumatol 18:663–676

    Article  PubMed  Google Scholar 

  10. Goldbach-Mansky R, Wilson M, Fleischmann R et al (2009) Comparison of Tripterygium wilfordii Hook F versus sulfasalazine in the treatment of rheumatoid arthritis: a randomized trial. Ann Intern Med 151(4):229–240, W49-51

    PubMed  Google Scholar 

  11. Kumari R, Uppal SS (2006) Prolonged remission in adult-onset Still's disease with etanercept. Clin Rheumatol 25(1):106–108

    Article  PubMed  Google Scholar 

  12. 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(2):466–467

    Article  CAS  PubMed  Google Scholar 

  13. Esdaile JM, Tannenbaum H, Hawkins D (1980) Adult Still's disease. Am J Med 68:825–830

    Article  CAS  PubMed  Google Scholar 

  14. Larson EB (1984) Adult Still's disease: evolution of a clinical syndrome and diagnosis, treatment and follow up of 17 patients. Medicine (Baltimore) 63:82–91

    CAS  Google Scholar 

  15. Wouters JM, van de Putte LB (1986) Adult onset Still's disease; clinical and laboratory features. Treatment and progress of 45 cases. Q J Med 61:1055–1065

    CAS  PubMed  Google Scholar 

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Correspondence to Wen Zhang.

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Kong, Xd., Xu, D., Zhang, W. et al. Clinical features and prognosis in adult-onset still's disease: a study of 104 cases. Clin Rheumatol 29, 1015–1019 (2010). https://doi.org/10.1007/s10067-010-1516-1

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  • DOI: https://doi.org/10.1007/s10067-010-1516-1

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