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Fulminant myocarditis with adult-onset Still’s disease: case-based review

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Abstract

Myocarditis has been reported as a life-threatening complication of adult-onset Still’s disease (AOSD), but fulminant myocarditis with AOSD is very rare. We hereby report a case of a 43-year-old female with fulminant myocarditis with AOSD. She had a refractory AOSD and cardiogenic shock with markedly elevated ferritin level up to 67,370 ng/mL. She was successfully treated with canakinumab and mechanical circulatory support (MCS) such as venoarterial extracorporeal membrane oxygenation and Impella CP. We also reviewed the previous cases of fulminant myocarditis with AOSD published from 1976 to December 2022, and only 8 cases of fulminant myocarditis with AOSD have been reported. The characteristics of these cases showed that the average age at presentation was 37.6 years (range 24–47 years). The time to myocarditis from the onset of AOSD ranged from 2 weeks to 2 years; however, most cases developed myocarditis within 1 year. Initial presenting symptoms included fever, dyspnea, chest pain, myalgia, rash, and sore throat. The median peak ferritin was 13,000 ng/mL. Left ventricular ejection fractions were not greater than 35%. Our case was the first reported case successfully treated with canakinumab and MCS. This review suggests that myocarditis may be an early phase of the complication in patients with AOSD, and the severity of AOSD may correlate with the severity of myocarditis. Canakinumab for AOSD and MCS for fulminant myocarditis may be one of the choices for overcoming the comorbidities.

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References

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

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  2. Gracia-Ramos AE, Contreras-Ortiz JA (2020) Myocarditis in adult-onset Still’s disease: case-based review. Clin Rheumatol 39(3):933–947

    Article  PubMed  Google Scholar 

  3. Parisi F, Paglionico A, Varriano V, Ferraccioli G, Gremese E (2017) Refractory adult-onset Still disease complicated by macrophage activation syndrome and acute myocarditis: a case report treated with high doses (8 mg/kg/d) of anakinra. Medicine (Baltimore) 96(24):e6656

    Article  PubMed  Google Scholar 

  4. Giacomelli R, Ruscitti P, Shoenfeld Y (2018) A comprehensive review on adult onset Still’s disease. J Autoimmun 93:24–36

    Article  PubMed  Google Scholar 

  5. Hintenberger R, Falkinger A, Danninger K, Pieringer H (2018) Cardiovascular disease in patients with autoinflammatory syndromes. Rheumatol Int 38(1):37–50

    Article  PubMed  Google Scholar 

  6. Gerfaud-Valentin M, Seve P, Iwaz J et al (2014) Myocarditis in adult-onset still disease. Medicine (Baltimore) 93(17):280–289

    Article  PubMed  Google Scholar 

  7. Veronese G, Ammirati E, Cipriani M, Frigerio M (2018) Fulminant myocarditis: characteristics, treatment, and outcomes. Anatol J Cardiol 19(4):279–286

    PubMed  PubMed Central  Google Scholar 

  8. 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  CAS  PubMed  Google Scholar 

  9. Colina M, Govoni M, Trotta F (2009) Fatal myocarditis in adult-onset Still disease with diffuse intravascular coagulation. Rheumatol Int 29(11):1355–1357

    Article  PubMed  Google Scholar 

  10. Jadhav P, Nanayakkara N (2009) Myocarditis in adult onset Stills disease. Int J Rheum Dis 12(3):272–274

    Article  PubMed  Google Scholar 

  11. Zhao DB, Dai SM, Liu XP, Xu H (2011) Interstitial inflammation in visceral organs is a pathologic feature of adult-onset Still’s disease. Rheumatol Int 31(7):923–927

    Article  PubMed  Google Scholar 

  12. Yamazoe M, Mizuno A, Suyama Y et al (2014) Endomyocardial biopsy and magnetic resonance imaging of acute myocarditis with adult-onset Still’s disease. Korean Circ J 44(6):437–440

    Article  PubMed  PubMed Central  Google Scholar 

  13. Savage E, Wazir T, Drake M, Cuthbert R, Wright G (2014) Fulminant myocarditis and macrophage activation syndrome secondary to adult-onset Still’s disease successfully treated with tocilizumab. Rheumatology (Oxford) 53(7):1352–1353

    Article  PubMed  Google Scholar 

  14. Gupta D, Jagani R, Mendonca S, Rathi KR (2016) Adult-onset Still’s disease with myocarditis and hemophagocytic lymphohistiocytosis: rare manifestation with fatal outcome. Indian J Pathol Microbiol 59(1):84–86

    PubMed  Google Scholar 

  15. Yamaguchi M, Ohta A, Tsunematsu T et al (1992) Preliminary criteria for classification of adult Still’s disease. J Rheumatol 19(3):424–430

    CAS  PubMed  Google Scholar 

  16. Liberati A, Altman DG, Tetzlaff J et al (2009) The PRISMA statement for reporting systematic reviews and meta-analyses of studies that evaluate healthcare interventions: explanation and elaboration. BMJ 339:b2700

    Article  PubMed  PubMed Central  Google Scholar 

  17. Alsaywid BS, Abdulhaq NM (2019) Guideline on writing a case report. Urol Ann 11:126–131

    Article  PubMed  PubMed Central  Google Scholar 

  18. Bodard Q, Langlois V, Guilpain P et al (2021) Cardiac involvement in adult-onset Still’s disease: manifestations, treatments and outcomes in a retrospective study of 28 patients. J Autoimmun 116:102541

    Article  CAS  PubMed  Google Scholar 

  19. Dall'Ara F, Frassi M, Tincani A, Airò P (2016) A retrospective study of patients with adult-onset Still’s disease: is pericarditis a possible predictor for biological disease-modifying anti-rheumatic drugs need? Clin Rheumatol 35(8):2117–2123

    Article  PubMed  Google Scholar 

  20. Dhimolea E (2010) Canakinumab. MAbs 2(1):3–13

    Article  PubMed  PubMed Central  Google Scholar 

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Ethics approval and consent to participate

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research commit- tee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards. Informed consent for publication has been obtained from the patient, in line with the COPE best practice guidelines.

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Authors and Affiliations

  1. Department of Cardiovascular Medicine, Chiba University Graduate School of Medicine, 1-8-1 Inohana, Chuo-ku, Chiba, 260-8670, Japan

    Ryohei Ono, Togo Iwahana, Shuji Toriumi, Kaoruko Aoki, Hirotoshi Kato & Yoshio Kobayashi

  2. Department of Allergy and Clinical Immunology, Chiba University Hospital, 1-8-1 Inohana, Chuo-ku, Chiba, 260-8677, Japan

    Kodai Kato, Masahiro Yasui, Yuzuho Nakagawa, Shunsuke Furuta & Hiroshi Nakajima

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  1. Ryohei Ono
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  2. Togo Iwahana
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  3. Shuji Toriumi
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  4. Kaoruko Aoki
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  5. Hirotoshi Kato
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  11. Yoshio Kobayashi
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Contributions

The case was diagnosed and followed up by RO, TI, ST, KA, HK, KK, MY, YN, and SF. RO conceived and planned the case report. TI, ST, KA, HK, KK, MY, YN, SF, HN, and YK contributed to the design of the work and interpretation of data. Material preparation, data collection, and analysis were performed by RO, TI, ST, KA, HK, KK, MY, YN, and SF. RO wrote the initial draft of the manuscript. TI, ST, KA, HK, KK, MY, YN, SF, HN, and YK revised and edited the manuscript. The final version was read, corrected, and approved by all authors, and all co-authors take full responsibility for the integrity and accuracy of all aspects of the work.

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Correspondence to Ryohei Ono.

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Ono, R., Iwahana, T., Toriumi, S. et al. Fulminant myocarditis with adult-onset Still’s disease: case-based review. Clin Rheumatol 42, 2507–2514 (2023). https://doi.org/10.1007/s10067-023-06648-y

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  • DOI: https://doi.org/10.1007/s10067-023-06648-y

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