Interleukin-1/6 Blockade for the Treatment of Severe Steroid-Refractory BNT162b2 Vaccine-Induced Adult-Onset Still’s Disease
KeywordsAdult-onset Still’s disease, myocarditis, COVID-19, vaccines, biological therapies
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.
Issue: 2022: Vol 9 No 8 (view)