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Fatal myocarditis in adult-onset Still disease with diffuse intravascular coagulation

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Adult-onset Still disease (AOSD) is a rare condition disease of unknown etiology, characterized by quotidian or double quotidian spiking fever, with an evanescent pink-salmon rash, arthritis and multi-organ involvement. Diagnosis is usually clinical and made after other diseases in the differential diagnosis are excluded. We herein report the case of a patient with a remarkable familial autoimmune background in whom adult Still disease started off with a diffuse intravascular coagulation, probably triggered by a macrophage activation syndrome, followed by an acute interstitial myocarditis, leading to a fatal complete atrioventricular block. This case highlights that AOSD represents a troubling condition and that it may suddenly get worse with life-threatening events.

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

    PubMed  CAS  Google Scholar 

  2. 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 

  3. Pouchot J, Sampalis JS, Beaudet F et al (1991) Adult Still’s disease: manifestations, disease course and outcome in 62 patients. Medicine 70:118–136. doi:10.1097/00005792-199103000-00004

    Article  PubMed  CAS  Google Scholar 

  4. Masson C, Le Loet X, Liote F et al (1995) Adult Still’s disease: part I. Manifestations and complications in sixty-five cases in France. Rev Rhum Engl Ed 62:748–757

    PubMed  CAS  Google Scholar 

  5. Dhote R, Simon J, Papo T et al (2003) Reactive hemophagocytic syndrome in adult systemic disease: report of twenty-six cases and literature review. Arthritis Rheum 49:633–639. doi:10.1002/art.11368

    Article  PubMed  Google Scholar 

  6. Esumi N, Ikushima S, Hibi S et al (1988) High serum ferritin level as a marker of malignant histiocytosis and virus-associated hemophagocytic syndrome. Cancer 61:2071–2076. doi:10.1002/1097-0142(19880515)61:10<2071::AID-NCR2820611023>3.0.CO;2-6

    Article  PubMed  CAS  Google Scholar 

  7. Bank I, Marboe CC, Redberg RF et al (1985) Myocarditis in adult Still’s disease. Arthritis Rheum 28:452–454. doi:10.1002/art.1780280415

    Article  PubMed  CAS  Google Scholar 

  8. Sachs RN, Talvard O, Lanfranchi J (1990) Myocarditis in adult Still’s disease. Int J Cardiol 27:377–380. doi:10.1016/0167-5273(90)90295-G

    Article  PubMed  CAS  Google Scholar 

  9. Hosaka S, Takashina N, Ishikawa A et al (1992) Adult Still’s disease with myocarditis and peritonitis. Int Med 31:812–815. doi:10.2169/internalmedicine.31.812

    Article  CAS  Google Scholar 

  10. Vandergheynst F, Gosset J, van de Borne P et al (2005) Myopericarditis revealing adult-onset Still’s disease. Acta Clin Belg 60:205–208

    PubMed  CAS  Google Scholar 

  11. Kristensen LE, Bartosik I (2006) Myocarditis in adult-onset Still’s disease despite significant immunosuppressive therapy. Scand J Rheumatol 35:330–331. doi:10.1080/03009740500499492

    Article  PubMed  CAS  Google Scholar 

  12. Kuek A, Weerakoon A, Ahmed K et al (2007) Adult-onset Still’s disease and myocarditis: successful treatment with intravenous immunoglobulin and maintenance of remission with etanercept. Rheumatology 46:1043–1044. doi:10.1093/rheumatology/kem066

    Article  PubMed  CAS  Google Scholar 

  13. Yang DH, Chang DM, Lai JH et al (2008) Etanercept as a rescue agent in patient with adult onset Still’s disease complicated with congestive heart failure. Rheumatol Int 29(1):95–98

    Article  PubMed  CAS  Google Scholar 

  14. Prieur AM (1996) Intravenous immunoglobulins in Still’s disease: still controversial, still unproven. J Rheumatol 23:797–800

    PubMed  CAS  Google Scholar 

  15. Fautrel B, Sibilia J, Mariette X et al (2005) Tumor necrosis factor alpha blocking agents in refractory adult Still’s disease: an observational study of 20 cases. Ann Rheum Dis 64:262–266. doi:10.1136/ard.2004.024026

    Article  PubMed  CAS  Google Scholar 

  16. Lequerré T, Quartier P, Rosellini D et al (2008) Interleukin-1 receptor antagonist (anakinra) treatment in patient with systemic-onset juvenile idiopathic arthritis or adult-onset Still disease: preliminary experience in France. Ann Rheum Dis 67:302–308. doi:10.1136/ard.2007.076034

    Article  PubMed  Google Scholar 

  17. Quartuccio L, De Vita S (2007) Interleukin 1 receptor antagonist therapy-induced thrombocytopenia in adult onset Still’s disease. J Rheumatol 34:892–893

    PubMed  Google Scholar 

  18. Ruiz PJ, Masliah E, Doherty TA et al (2007) Cardiac death in a patient with adult-onset Still’s disease treated with the interleukin 1 receptor inhibitor anakinra. Ann Rheum Dis 66:422–423. doi:10.1136/ard.2006.060541

    Article  PubMed  CAS  Google Scholar 

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Correspondence to Matteo Colina.

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Colina, M., Govoni, M. & Trotta, F. Fatal myocarditis in adult-onset Still disease with diffuse intravascular coagulation. Rheumatol Int 29, 1355–1357 (2009).

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