Flare of adult-onset Still’s disease following mRNA COVID-19 vaccination: a case report and review of literature

Abstract

The upheaval caused by the coronavirus disease 2019 (COVID-19) pandemic has allowed to large population to use new vaccines urgently. Although vaccine development programs and available epidemiological data reassure us, there are concerns about specific risks associated with vaccinations in patients with autoimmune-autoinflammatory diseases. These patients have the potential to decrease humoral and cellular immune responses caused by biologic agents and develop an acute flare of underlying disease following vaccination. We herein present a rare case of a 49-year-old female with a flare of adult-onset Still’s disease (AOSD) after the first dose of BNT162b2 mRNA COVID-19 vaccination. She had been diagnosed with AOSD 7 years earlier and had achieved remission with tocilizumab. This patient came to the emergency room with fever and nausea that occurred 4 days after the first vaccination. Based on laboratory results and clinical manifestations, we suspected AOSD flare and was treated with steroid pulse therapy. In this report, we also discuss possible mechanisms linking vaccination with a flare of AOSD. Considering the close time relationship between COVID-19 vaccinations and a flare of AOSD, physicians should be aware of adverse events from this new vaccination and evaluate the benefits and risks of vaccination for each patient. Key Points • COVID-19 vaccination may cause an AOSD flare in patients who are in remission with tocilizumab.

Diagnosis and treatment of adult-onset Still’s disease: a concise summary of the German society of rheumatology S2 guideline

“…In certain cases, genetic studies may be useful to exclude hereditary autoinflammatory diseases, but do not show sufficient diagnostic precision to be routinely recommended in AOSD work-up [12,31,42,97,189,211]. From a diagnostic perspective, it is noteworthy that flares or primary manifestations of AOSD have been noted in temporal relation to COVID vaccinations during the course of the current pandemic [99,123,145,159,172,212]. Concerning therapeutic options, the board decided against phrasing a recommendation for or against tumor necrosis factor (TNF)-blocking agents.…”

New-onset Adult-onset Still's Disease Following COVID-19 Vaccination: Three Case Reports and a Literature Review

“…We herein report three cases of AOSD that developed following vaccination with mRNA COVID-19 vaccines (two with BNT162b2 and one with mRNA-1273). To our knowledge, 10 other cases of new-onset (cases 1-10 in the Table ) and relapse of AOSD (cases 14-17 in Table ) have been reported following COVID-19 vaccination ( 13 - 23 ). Seven of those 10 patients developed symptoms 5-21 days after the first or second dose of the COVID-19 vaccine ( Table ).…”

“…Previous studies have reported AOSD that developed after influenza vaccination; since 2021, cases of new-onset AOSD and relapsed immune-mediated disease have also occurred after COVID-19 vaccination (7,8,10,(13)(14)(15)(16)(17)(18)(19)(20)(21)(22)(23). There are two main types of COVID-19 vaccine formulations: mRNA encoding the SARS-CoV-2 spike protein (S) encapsulated in lipid nanoparticles or adenovirus (AdV) vectors encoding the S protein (35).…”

New-Onset Arthritis Following COVID-19 Vaccination: A Systematic Review of Case Reports

“…SARS-CoV-2 infection could also induce cytokine storms driven by IL-6, IL-1α, IL-1β, and TNF-α [ 42 ], and the pathogenesis of both is similar. Multiple case reports have shown that vaccination against COVID-19 can induce a strong immune response [ 43 , 44 ]. However, it is not possible to distinguish whether an adverse effect of vaccination is the induction of new-onset AOSD or whether it is actually a relapse of existing AOSD that plays a major part in this mechanism.…”