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Case Report: Adult-Onset Still’s Disease with Complications

Cristina Romaniello, DO, & Caitlin Kesari, MD  |  Issue: February 2023  |  February 14, 2023

Caitlin Kesari, MD, is a board-certified rheumatologist practicing in Columbus, Ohio. She completed her rheumatology fellowship at the University of Cincinnati Medical Center and Cincinnati VA.

References

  1. Akintayo RO, Adelowo O. Adult-onset Still’s disease in a Nigerian woman. BMJ Case Rep. 2015 Jul 6;2015:bcr2015210789.
  2. Bae CB, Jung JY, Kim HA, et al. Reactive hemophagocytic syndrome in adult-onset Still disease: Clinical features, predictive factors, and prognosis in 21 patients. Medicine (Baltimore). 2015 Jan;94(4):e451.
  3. Fautrel B. Adult-onset Still disease. Best Pract Res Clin Rheumatol. 2008 Oct;22(5):773–792.
  4. Fautrel B, Zing E, Golmard JL, et al. Proposal for a new set of classification criteria for adult-onset Still disease. Medicine (Baltimore). 2002 May;81(3):194–200.
  5. Gao Q, Yuan Y, Wang Y, et al. Clinical characteristics of macrophage activation syndrome in adult-onset Still’s disease. Clin Exp Rheumatol. 2021 Sep–Oct;39 Suppl 132(5):59–66.
  6. Giacomelli R, Ruscitti P, Shoenfeld Y. A comprehensive review on adult onset Still’s disease. J Autoimmun. 2018 Sep;93:24–36.
  7. Giampietro C, Ridene M, Lequerre T, et al. Anakinra in adult-onset Still’s disease: long-term treatment in patients resistant to conventional therapy. Arthritis Care Res (Hoboken). 2013 May;65(5):822–826.
  8. Gopalarathinam R, Orlowsky E, Kesavalu R, et al. Adult onset Still’s disease: A review on diagnostic workup and treatment options. Case Rep Rheumatol. 2016;2016:6502373.
  9. Franchini S, Dagna L, Salvo F, et al. Efficacy of traditional and biologic agents in different clinical phenotypes of adult-onset Still’s disease. Arthritis Rheum. 2010 Aug;62(8):2530–2535.
  10. Kedor C, Listing J, Zernicke J, et al. Canakinumab for treatment of adult-onset Still’s disease to achieve reduction of arthritic manifestation (CONSIDER): phase II, randomised, double-blind, placebo-controlled, multicentre, investigator-initiated trial. Ann Rheum Dis. 2020 Aug;79(8):1090–1097.
  11. Magadur-Joly G, Billaud E, Barrier JH, et al. Epidemiology of adult Still’s disease: estimate of the incidence by a retrospective study in west France. Ann Rheum Dis. 1995 Jul;54(7):587–590.
  12. Nordström D, Knight A, Luukkainen R, et al. Beneficial effect of interleukin 1 inhibition with anakinra in adult-onset Still’s disease. An open, randomized, multicenter study. J Rheumatol. 2012 Oct;39(10):2008–2011.
  13. Yamaguchi M, Ohta A, Tsunematsu T, et al. Preliminary criteria for classification of adult Still’s disease. J Rheumatol. 1992 Mar; 19(3):424–430.
  14. Efthimiou P, Kontzias A, Hur P, et al. Adult-onset Still’s disease in focus: Clinical manifestations, diagnosis, treatment, and unmet needs in the era of targeted therapies. Semin Arthritis Rheum. 2021 Aug;51(4):858–874.

Editor’s note: The African continent is full of diversity in every aspect. Although we were unable to ascertain precisely which country or region the patient had immigrated from, the key learning point remains: Immune dysfunction is often overlooked in those living in Africa and among African diaspora communities. Clinicians should maintain a high index of suspicion for these conditions.

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Filed under:ConditionsOther Rheumatic Conditions Tagged with:adult Still's Diseasecase reportmacrophage activation syndrome

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