Interleukin-1/6 Blockade for the Treatment of Severe Steroid-Refractory BNT162b2 Vaccine-Induced Adult-Onset Still’s Disease
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’s disease (AOSD) with salmon-pink flagellate erythema, polyarthritis,...
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| Format: | Article |
| Language: | English |
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SMC MEDIA SRL
2022-08-01
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| Series: | European Journal of Case Reports in Internal Medicine |
| Subjects: | |
| Online Access: | https://www.ejcrim.com/index.php/EJCRIM/article/view/3469 |
| _version_ | 1811262242042150912 |
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| author | Benjamin Hugues Hakim Ben Amer Floriane Bril Matthieu Groh Florent Huang |
| author_facet | Benjamin Hugues Hakim Ben Amer Floriane Bril Matthieu Groh Florent Huang |
| author_sort | Benjamin Hugues |
| collection | DOAJ |
| description | 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’s disease (AOSD) with salmon-pink flagellate erythema, polyarthritis, a sore throat, myocarditis and haemophagocytic lymphohistiocytosis after receiving two doses of the BNT162b2 vaccine (Pfizer®, BioNTech®). 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’s disease. At a population level, the favourable benefit/risk ratio of COVID-19 vaccination remains indisputable. |
| first_indexed | 2024-04-12T19:21:38Z |
| format | Article |
| id | doaj.art-7462aa9438944352ba067cc4b99f13b6 |
| institution | Directory Open Access Journal |
| issn | 2284-2594 |
| language | English |
| last_indexed | 2024-04-12T19:21:38Z |
| publishDate | 2022-08-01 |
| publisher | SMC MEDIA SRL |
| record_format | Article |
| series | European Journal of Case Reports in Internal Medicine |
| spelling | doaj.art-7462aa9438944352ba067cc4b99f13b62022-12-22T03:19:35ZengSMC MEDIA SRLEuropean Journal of Case Reports in Internal Medicine2284-25942022-08-0110.12890/2022_0034693004Interleukin-1/6 Blockade for the Treatment of Severe Steroid-Refractory BNT162b2 Vaccine-Induced Adult-Onset Still’s DiseaseBenjamin Hugues0Hakim Ben Amer1Floriane Bril2Matthieu Groh3Florent Huang4Service de Médecine Interne, Hôpital Foch, Suresnes, FranceService de Cardiologie, Hôpital Foch, Suresnes, FranceService de Cardiologie, Hôpital Foch, Suresnes, FranceService de Médecine Interne, Hôpital Foch, Suresnes, FranceService de Cardiologie, Hôpital Foch, Suresnes, FranceIntroduction: 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’s disease (AOSD) with salmon-pink flagellate erythema, polyarthritis, a sore throat, myocarditis and haemophagocytic lymphohistiocytosis after receiving two doses of the BNT162b2 vaccine (Pfizer®, BioNTech®). 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’s disease. At a population level, the favourable benefit/risk ratio of COVID-19 vaccination remains indisputable.https://www.ejcrim.com/index.php/EJCRIM/article/view/3469adult-onset still’s diseasemyocarditiscovid-19vaccines biological therapies |
| spellingShingle | Benjamin Hugues Hakim Ben Amer Floriane Bril Matthieu Groh Florent Huang Interleukin-1/6 Blockade for the Treatment of Severe Steroid-Refractory BNT162b2 Vaccine-Induced Adult-Onset Still’s Disease European Journal of Case Reports in Internal Medicine adult-onset still’s disease myocarditis covid-19 vaccines biological therapies |
| title | Interleukin-1/6 Blockade for the Treatment of Severe Steroid-Refractory BNT162b2 Vaccine-Induced Adult-Onset Still’s Disease |
| title_full | Interleukin-1/6 Blockade for the Treatment of Severe Steroid-Refractory BNT162b2 Vaccine-Induced Adult-Onset Still’s Disease |
| title_fullStr | Interleukin-1/6 Blockade for the Treatment of Severe Steroid-Refractory BNT162b2 Vaccine-Induced Adult-Onset Still’s Disease |
| title_full_unstemmed | Interleukin-1/6 Blockade for the Treatment of Severe Steroid-Refractory BNT162b2 Vaccine-Induced Adult-Onset Still’s Disease |
| title_short | Interleukin-1/6 Blockade for the Treatment of Severe Steroid-Refractory BNT162b2 Vaccine-Induced Adult-Onset Still’s Disease |
| title_sort | interleukin 1 6 blockade for the treatment of severe steroid refractory bnt162b2 vaccine induced adult onset still s disease |
| topic | adult-onset still’s disease myocarditis covid-19 vaccines biological therapies |
| url | https://www.ejcrim.com/index.php/EJCRIM/article/view/3469 |
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