{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,8,23]],"date-time":"2025-08-23T00:06:31Z","timestamp":1755907591130,"version":"3.44.0"},"reference-count":11,"publisher":"SMC Media","issue":"8","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"abstract":"<jats:p>Introduction: Several immune-mediated side effects have been reported with COVID-19 vaccines, including myocarditis. Case description: A 27-year-old woman with a past medical history of mild COVID-19, developed adult-onset Still\u2019s disease (AOSD) with salmon-pink flagellate erythema, polyarthritis, a sore throat, myocarditis and haemophagocytic lymphohistiocytosis after receiving two doses of the BNT162b2 vaccine (Pfizer\u00ae, BioNTech\u00ae). Despite the initial efficacy of high-dose pulses of methylprednisolone, inflammatory markers rose as soon as de-escalation of corticosteroids was attempted, warranting initiation of biologics targeting the interleukin (IL)-1\/6 axis, which allowed sustained remission of the disease despite withdrawal of corticosteroids. Discussion: To our knowledge, this is the first case of AOSD with both haemophagocytic lymphohistiocytosis and cardiac magnetic resonance imaging-proven myocarditis triggered by COVID-19 vaccination, successfully treated with steroids and biologics targeting the IL-1\/IL-6 axis. The pathophysiological process by which COVID-19 vaccination can lead to AOSD is still unknown, although it has been reported that the spike protein may act as a pathogen-associated molecular pattern and thus induce an overproduction of pro-inflammatory cytokines of the innate immune system (e.g., IL-1, IL-6 or IL-18). Conclusion: Targeting the IL-1\/6 axis is effective for the treatment of severe steroid-refractory BNT162b2 vaccine-induced adult-onset Still\u2019s disease. At a population level, the favourable benefit\/risk ratio of COVID-19 vaccination remains indisputable.<\/jats:p>","DOI":"10.12890\/2022_003469","type":"journal-article","created":{"date-parts":[[2022,8,29]],"date-time":"2022-08-29T04:09:22Z","timestamp":1661746162000},"source":"Crossref","is-referenced-by-count":1,"title":["Interleukin-1\/6 Blockade for the Treatment of Severe Steroid-Refractory BNT162b2 Vaccine-Induced Adult-Onset Still\u2019s Disease"],"prefix":"10.12890","volume":"9","author":[{"given":"Benjamin","family":"Hugues","sequence":"first","affiliation":[{"name":"Service de M\u00e9decine Interne, H\u00f4pital Foch, Suresnes, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hakim","family":"Ben Amer","sequence":"first","affiliation":[{"name":"Service de Cardiologie, H\u00f4pital Foch, Suresnes, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Floriane","family":"Bril","sequence":"first","affiliation":[{"name":"Service de Cardiologie, H\u00f4pital Foch, Suresnes, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Matthieu","family":"Groh","sequence":"first","affiliation":[{"name":"Service de M\u00e9decine Interne, H\u00f4pital Foch, Suresnes, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Florent","family":"Huang","sequence":"first","affiliation":[{"name":"Service de Cardiologie, H\u00f4pital Foch, Suresnes, France"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"10397","published-online":{"date-parts":[[2022,8,29]]},"reference":[{"key":"10.12890\/2022_003469_ref1","doi-asserted-by":"crossref","unstructured":"<li>Barda N, Dagan N, Ben-Shlomo Y, Kepten E, Waxman J, Ohana R, et al. Safety of the BNT162b2 mRNA Covid-19 vaccine in a nationwide setting. <em>N Engl J Med<\/em> 2021 <strong>16<\/strong>;385(12):1078\u20131090. <\/li>","DOI":"10.1056\/NEJMoa2110475"},{"key":"10.12890\/2022_003469_ref2","doi-asserted-by":"crossref","unstructured":"<li>Fardet L, Galicier L, Lambotte O, Marzac C, Aumont C, Chahwan D, et al. Development and validation of the HScore, a score for the diagnosis of reactive hemophagocytic syndrome. <em>Arthritis Rheumatol<\/em> 2014;<strong>66<\/strong>(9):2613\u20132620. <\/li>","DOI":"10.1002\/art.38690"},{"key":"10.12890\/2022_003469_ref3","unstructured":"<li>Yamaguchi M, Ohta A, Tsunematsu T, Kasukawa R, Mizushima Y, Kashiwagi H, et al. Preliminary criteria for classification of adult Still\u2019s disease. <em>J Rheumatol<\/em> 1992;<strong>19<\/strong>(3):424\u2013430. <\/li>"},{"key":"10.12890\/2022_003469_ref4","doi-asserted-by":"crossref","unstructured":"<li>Fautrel B, Zing E, Golmard JL, Le Moel G, Bissery A, Rioux C, et al. Proposal for a new set of classification criteria for adult-onset still disease. <em>Medicine (Baltimore)<\/em> 2002;<strong>81<\/strong>(3):194\u2013200. <\/li>","DOI":"10.1097\/00005792-200205000-00003"},{"key":"10.12890\/2022_003469_ref5","doi-asserted-by":"crossref","unstructured":"<li>Feist E, Mitrovic S, Fautrel B. Mechanisms, biomarkers and targets for adult-onset Still\u2019s disease. <em>Nat Rev Rheumatol<\/em> 2018;<strong>14<\/strong>(10):603\u2013618. <\/li>","DOI":"10.1038\/s41584-018-0081-x"},{"key":"10.12890\/2022_003469_ref6","doi-asserted-by":"crossref","unstructured":"<li>Leone F, Cerasuolo PG, Bosello SL, Verardi L, Fiori E, Cocciolillo F, et al. Adult-onset Still\u2019s disease following COVID-19 vaccination. <em>Lancet Rheumatol<\/em> 2021;<strong>3<\/strong>(10):e678\u2013680. <\/li>","DOI":"10.1016\/S2665-9913(21)00218-6"},{"key":"10.12890\/2022_003469_ref7","doi-asserted-by":"crossref","unstructured":"<li>Magliulo D, Narayan S, Ue F, Boulougoura A, Badlissi F. Adult-onset Still\u2019s disease after mRNA COVID-19 vaccine. <em>Lancet Rheumatol<\/em> 2021;<strong>3<\/strong>(10):e680\u2013682. <\/li>","DOI":"10.1016\/S2665-9913(21)00219-8"},{"key":"10.12890\/2022_003469_ref8","doi-asserted-by":"crossref","unstructured":"<li>Muench F, Krusche M, Sander LE, Rose T, Burmester GR, Schneider U. Macrophage activation syndrome in a patient with adult-onset Still\u2019s disease following first COVID-19 vaccination with BNT162b2. <em>BMC Rheumatol<\/em> 2021;<strong>5<\/strong>(1):60. <\/li>","DOI":"10.1186\/s41927-021-00237-9"},{"key":"10.12890\/2022_003469_ref9","doi-asserted-by":"crossref","unstructured":"<li>Jeon YH, Lim DH, Choi SW, Choi SJ. A flare of Still\u2019s disease following COVID-19 vaccination in a 34-year-old patient. <em>Rheumatol Int<\/em> 2022;<strong>42<\/strong>(4):743\u2013748.<\/li>","DOI":"10.1007\/s00296-021-05052-6"},{"key":"10.12890\/2022_003469_ref10","doi-asserted-by":"crossref","unstructured":"<li>Pardi N, Hogan MJ, Porter FW, Weissman D. mRNA vaccines - a new era in vaccinology. <em>Nat Rev Drug Discov<\/em> 2018;<strong>17<\/strong>(4):261\u2013279. <\/li>","DOI":"10.1038\/nrd.2017.243"},{"key":"10.12890\/2022_003469_ref11","doi-asserted-by":"crossref","unstructured":"<li>Mehta P, McAuley DF, Brown M, Sanchez E, Tattersall RS, Manson JJ, et al. COVID-19: consider cytokine storm syndromes and immunosuppression. <em>Lancet<\/em> 2020;<strong>395<\/strong>(10229):1033\u20131034.<\/li>","DOI":"10.1016\/S0140-6736(20)30628-0"}],"container-title":["European Journal of Case Reports in Internal Medicine"],"original-title":[],"link":[{"URL":"https:\/\/www.ejcrim.com\/index.php\/EJCRIM\/article\/download\/3469\/3203","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/www.ejcrim.com\/index.php\/EJCRIM\/article\/download\/3469\/3204","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/www.ejcrim.com\/index.php\/EJCRIM\/article\/download\/3469\/3204","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,8,22]],"date-time":"2025-08-22T09:23:48Z","timestamp":1755854628000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.ejcrim.com\/index.php\/EJCRIM\/article\/view\/3469"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,8,29]]},"references-count":11,"journal-issue":{"issue":"8","published-online":{"date-parts":[[2022]]}},"URL":"https:\/\/doi.org\/10.12890\/2022_003469","relation":{"has-manifestation":[{"id-type":"other","id":"1-89-3469-g3203","asserted-by":"subject"},{"id-type":"other","id":"1-89-3469-g3204","asserted-by":"subject"}],"is-part-of":[{"id-type":"other","id":"1-89","asserted-by":"subject"}]},"ISSN":["2284-2594"],"issn-type":[{"type":"electronic","value":"2284-2594"}],"subject":[],"published":{"date-parts":[[2022,8,29]]}}}