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, 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.

LEARNING POINTS

Adult-onset Still's disease can be triggered by the BNT162b2 COVID-19 vaccine.Biologics targeting the interleukin-1/6 axis should be considered early in the course of vaccine-induced adult-onset Still's disease, especially when steroids do not effectively curb the disease.