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Ten years of clinical experience with adult onset Still’s disease: is the outcome improving?

Clinical Rheumatology volume 26, pages 1055–1060 (2007)Cite this article

Abstract

This study aims to report on the clinical and laboratory picture and the disease course and outcome in patients having adult onset Still’s disease (AOSD), to briefly review existing literature on the subject, and to compare our findings with those previously reported. Results are reported for 28 patients with AOSD satisfying the preliminary criteria of Yamaguchi et al. seen in a teaching hospital over the last 10 years. A high percent of the patients with AOSD were women. The mean (+SD) age at disease onset was 27.8 (+8.4) years. We found fever in 100%, rash in 85%, arthritis in 64%, lymphadenopathy in 60%, splenomegaly in 57%, hepatomegaly in 35%, pleural effusion in 17.9%, and pericardial effusion in 3.6% of our patients. Leukocytosis was present in 96% of the patients, a normochromic, normocytic anemia in 54%, and an elevated erythrocyte sedimentation rate (ESR) in all. Serum ferritin levels were raised in 89% of the patients. The mean follow-up of the patients was 3.72 + 2.46 years. The mean delay in diagnosis was 7.32 + 18.0 months. The mean time to enter remission was 9.7 months. Self-limited, intermittent, and chronic disease course was seen in 14.3, 57.1, and 28.6% of patients, respectively. The outcome was good in about 89% of patients, and mortality was nil. No particular clinical or laboratory variable was found to predict the subsequent disease course and outcome in our patients. On comparing our data with important previous series, we found a higher percentage of women and of patients presenting in the age group 16–35 years, a lower frequency of arthritis and pericardial effusion, and some other notable differences. Importantly, the disease course was benign, probably as an outcome of heightened awareness and less diagnostic delay than in the past, allowing for early, aggressive, and appropriate treatment. It is concluded that AOSD is now a relatively benign disease if diagnosed early and treated appropriately.

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References

  1. Still GF (1897) On a form of chronic joint disease in children. Med Chir Trans 80:47

    Google Scholar 

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

    PubMed  CAS  Article  Google Scholar 

  3. Al-Arfaj AS, Al-Saleh S (2001) Adult-onset Still’s disease in Saudi Arabia. Clin Rheumatol 20:197–200

    PubMed  Article  CAS  Google Scholar 

  4. Chen DY, Lan JL, Hsieh TY, Chen YH (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 

  5. Ohta A, Yamaguchi M, Kaneoka H, Nagayoshi T, Hiida M (1987) Adult Still’s disease: review of 228 cases from the literature. J Rheumatol 14:1139–1146

    PubMed  CAS  Google Scholar 

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

    PubMed  CAS  Google Scholar 

  7. Pay S, Turkcapar N, Kalyoncu M, Simsek I, Beyan E, Ertenli I et al (2006) A multicenter study of patients with adult-onset Still’s disease compared with systemic juvenile idiopathic arthritis. Clin Rheumatol 25(5):639–644

    PubMed  Article  Google Scholar 

  8. Pouchot J, Sampalis JS, Beaudet F, Carette S, Decary F, Salusinsky-Sternbach M et al (1991) Adult Still’s disease: manifestations, disease course, and outcome in 62 patients. Medicine (Baltimore) 70:118–136

    CAS  Google Scholar 

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

    PubMed  Article  CAS  Google Scholar 

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

    PubMed  CAS  Google Scholar 

  11. Andres E, Kurtz JE, Perrin AE, Pflumio F, Ruellan A, Goichot B et al (2003) Retrospective monocentric study of 17 patients with adult Still’s disease, with special focus on liver abnormalities. Hepato-gastroenterology 50:192–195

    PubMed  Google Scholar 

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

    PubMed  Article  CAS  Google Scholar 

  13. Garcia-Porrua C, Gonzalez-Gay MA, Crespo F, Gonzalez-Juanatey C (2000) Adult onset Still’s disease in Catalonia, Spain. J Rheumatol 27:280–281

    PubMed  CAS  Google Scholar 

  14. Liu Y, Huang CB, Cai XH (1993) Adult onset Still’s disease: review of 50 cases and evaluation of diagnostic criteria. Zhonghua Nei Ke Za Zhi 32:603–606

    PubMed  CAS  Google Scholar 

  15. Mert A, Ozaras R, Tabak F, Bilir M, Ozturk R, Ozdogan H et al (2003) Fever of unknown origin: a review of 20 patients with adult-onset Still’s disease. Clin Rheumatol 22:89–93

    PubMed  Article  CAS  Google Scholar 

  16. Yamaguchi M, Ohta A, Tsunematsu T, Kasukawa R, Mizushima Y, Kashiwagi H et al (1992) Preliminary criteria for classification of adult Still’s disease. J Rheumatol 19:424–430

    PubMed  CAS  Google Scholar 

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

    PubMed  Article  Google Scholar 

  18. Nara H, Mimori A (1999) Adult Still’s disease. Nippon Rinsho 57:384–387

    PubMed  CAS  Google Scholar 

  19. van de Putte LB, Wouters JM (1991) Adult-onset Still’s disease. Bailliere’s Clin Rheumatol 5:263–275

    Article  Google Scholar 

  20. Masson C, Le Loet X, Liote F, Dubost JJ, Boissier MC, Perroux-Goumy L et al (1996) Comparative study of 6 types of criteria in adult Still’s disease. J Rheumatol 23:495–497

    PubMed  CAS  Google Scholar 

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

    PubMed  Article  CAS  Google Scholar 

  22. Mok CC, Lau CS, Wong RW (1998) Clinical characteristics, treatment, and outcome of adult onset Still’s disease in southern Chinese. J Rheumatol 25:2345–2351

    PubMed  CAS  Google Scholar 

  23. Wakai K, Ohta A, Tamakoshi A, Ohno Y, Kawamura T, Aoki R 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

    PubMed  CAS  Google Scholar 

  24. Akritidis N, Giannakakis I, Giouglis T (1996) Ferritin levels and response to treatment in patients with Adult Still’s disease. J Rheumatol 23:201–202

    PubMed  CAS  Google Scholar 

  25. Akritidis N, Giannakakis Y, Sakkas L (1997) Very high serum ferritin levels in adult-onset Still’s disease. Br J Rheumatol 36:608–609

    PubMed  Article  CAS  Google Scholar 

  26. Nguyen KH, Weisman MH (1997) Severe sore throat as a presenting symptom of adult onset Still’s disease: a case series and review of the literature. J Rheumatol 24:592–597

    PubMed  CAS  Google Scholar 

  27. Elkon KB, Hughes GR, Bywaters EG, Ryan PF, Inman RD, Bowley NB et al (1982) Adult-onset Still’s disease. Twenty-year followup and further studies of patients with active disease. Arthritis Rheum 25:647–654

    PubMed  Article  CAS  Google Scholar 

  28. 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

    PubMed  CAS  Google Scholar 

  29. 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 

  30. Nkoghe D, Demonty J, Leonard P, Nnegue S, Moutschen M, Kaye O (2002) Still’s disease in the adult. Rev Med Liege 57:213–219

    PubMed  CAS  Google Scholar 

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

    PubMed  Article  CAS  Google Scholar 

  32. Fautrel B, Sibilia J, Mariette X, Combe B (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

    PubMed  Article  CAS  Google Scholar 

  33. Kokkinos A, Iliopoulos A, Greka P, Efthymiou A, Katsilambros N, Sfikakis PP (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

    PubMed  Article  Google Scholar 

  34. Benucci M, Li GF, Del RA, Manfredi M (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 

  35. Arlet JB, LE Thi HD, Marinho A, Amoura Z, Wechsler B, Papo T et al (2006) Reactive haemophagocytic syndrome in adult onset Still’s disease: report of 6 patients and review of the literature. Ann Rheum Dis (in press). DOI 10.1136/ard.2005.046904

  36. Fietta P, Manganelli P (2003) The hemophagocytic syndrome (macrophage activation syndrome). Minerva Med 94:19–27

    PubMed  CAS  Google Scholar 

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Affiliations

  1. Rheumatology Division, Department of Medicine, Mubarak Al-Kabeer Hospital, Jabriya, Kuwait

    Sukhbir Singh Uppal, Moudhi Al-Mutairi, Sawsan Hayat & Anand Malaviya

  2. Department of Medicine, Faculty of Medicine, Kuwait University, Kuwait, Kuwait

    Sukhbir Singh Uppal, Mini Abraham & Anand Malaviya

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  1. Sukhbir Singh Uppal
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  2. Moudhi Al-Mutairi
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  3. Sawsan Hayat
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Correspondence to Sukhbir Singh Uppal.

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Uppal, S.S., Al-Mutairi, M., Hayat, S. et al. Ten years of clinical experience with adult onset Still’s disease: is the outcome improving?. Clin Rheumatol 26, 1055–1060 (2007). https://doi.org/10.1007/s10067-006-0440-x

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  • DOI: https://doi.org/10.1007/s10067-006-0440-x

Keywords

  • Adult
  • Adult onset Still’s disease
  • Clinical
  • Outcome
  • Still’s disease