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Adult-Onset Still's Disease

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Abstract

Adult-onset Still's disease (AOSD) was described originally by Eric Bywaters in 1971. AOSD closely resembles systemic-onset juvenile idio-pathic arthritis, a pediatric disorder known originally as Still's disease. An intensely inflammatory, multiorgan system disorder characterized by a polyarthritis, high spiking fevers, and an evanescent, salmon-colored rash (Figs. 3.1a–c). The rash is described as small macules that disappear during the night but reappear the next day, usually coincident with a fever spike. Peripheral joint involvement can be fleeting but also may settle into a refractory, destructive arthritis marked by a tendency in some joints for fusion. AOSD is typically seronegative. Patients do not have rheumatoid factor or antibodies to cyclic citrullinated peptides. Other common features are leukocytosis, thrombocy-tosis, elevations of the serum hepatic aminotransferase concentrations, splenomegaly, and serositis. Pharyngitis is often the initial symptom.

Keywords

  • Juvenile Idiopathic Arthritis
  • Interstitial Lung Disease
  • Familial Mediterranean Fever
  • Juvenile Rheumatoid Arthritis
  • Serum Ferritin Level

These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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Fig. 3.1

References

  • Allantaz F, Chaussabel D, Banchereau J, Pascual V. Microarray-based identification of novel biomarkers in IL-1-medicated diseases. Curr Opin Immunol 2007; 19:623–632

    PubMed  CrossRef  CAS  Google Scholar 

  • Aptekar RG, Decker JL, Bujak JS, Wolff SM. Adult onset juvenile rheumatoid arthritis. Arthritis Rheum 1973; 16:715–718

    PubMed  CrossRef  CAS  Google Scholar 

  • Bresnihan B. Cytokine neutralizers: IL-1 inhibitors. In: Hochberg MC, Silman AJ, Smolen JS, Weinblatt ME, Weisman MH (eds) Rheumatology (4th edn). Edinburgh, UK, Mosby, 2008, pp. 495–500

    Google Scholar 

  • Bywaters, EGL. Still's disease in the adult. Ann Rheum Dis 1971; 30:121–132

    PubMed  CrossRef  CAS  Google Scholar 

  • Esdaile J. Adult Still's disease. In: Hochberg MC, Silman AJ, Smolen JS, Weinblatt ME, Weisman MH (eds) Rheumatology (4th edn). Edinburgh, UK, Mosby, 2008, pp. 785–792

    Google Scholar 

  • Fautrel B, Sibilia J, Mariettte X, et al Tumor necrosis factor alpha blocking agents in refractory adult Still's disease: An observational study of 20 cases. Ann Rheum Dis 2005; 64:262–266

    PubMed  CrossRef  CAS  Google Scholar 

  • Fitzgerald AA, LeClercq SA, Yan A, et al Rapid responses to anakinra in patients with refractory adult-onset Still's disease. Arthritis Rheum 2005; 52:1794–1803

    PubMed  CrossRef  CAS  Google Scholar 

  • 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

    PubMed  CrossRef  CAS  Google Scholar 

  • Nguyen KH, Weisman MH. Severe sore throat as a presenting symptom of adult-onset still's disease: A case series and review of the literature. J Rheumatol 1997; 24:592–597

    PubMed  CAS  Google Scholar 

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Weisman, M.H. (2009). Adult-Onset Still's Disease. In: Stone, J.H. (eds) A Clinician's Pearls and Myths in Rheumatology. Springer, London. https://doi.org/10.1007/978-1-84800-934-9_3

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  • DOI: https://doi.org/10.1007/978-1-84800-934-9_3

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