A flare of Still’s disease following COVID-19 vaccination in a 34-year-old patient

Jeon, Young Hoon; Lim, Doo-Ho; Choi, Seung Won; Choi, Su Jin

doi:10.1007/s00296-021-05052-6

Cited by 19 publications

(15 citation statements)

References 26 publications

(27 reference statements)

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“…Several observational studies reported that the incidence and profile of adverse events of these vaccines in this patient population have been similar to the general population and severe AEs have been rarely observed [ 17 – 25 ]. On the other hand, post-vaccination flares in several patients with RD as well as new-onset RD in previously healthy individuals have been documented in a number of case series and registries [ 26 – 30 ]. It has to be noted that, most of the published data relate with classical forms of RD, and there is limited information with rare inflammatory disorders such as Behçet’s syndrome (BS) and familial Mediterranean fever (FMF).…”

Section: Introductionmentioning

confidence: 99%

Safety of SARS-CoV-2 vaccination in patients with Behcet’s syndrome and familial Mediterranean fever: a cross-sectional comparative study on the effects of M-RNA based and inactivated vaccine

et al. 2022

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Most of the published data relate to classical forms of rheumatic diseases (RD) and information on rare inflammatory disorders such as Behçet’s syndrome (BS) and familial Mediterranean fever (FMF) is limited. We studied the frequency of side effects and disease flares after COVID-19 vaccination with either Pfizer/BioNTech or Sinovac/CoronaVac in 256 patients with BS, 247 with FMF, and 601 with RD. Telephone interviews were conducted using a questionnaire survey in a cross-sectional design in patients with BS, FMF, and RD followed by a single university hospital. Study participants were vaccinated either with CoronaVac (BS:109, FMF: 90, and RD: 343,) or BioNTech (BS: 147, FMF: 157 and RD: 258). The majority have received double dose (BS: 94.9%, FMF 92.3% and RD: 86.2%). BioNTech ensured a significantly better efficacy than CoronaVac against COVID-19 in all patient groups (BS: 1.4% vs 10.1%; FMF: 3.2% vs 12.2%, RD:2.7% vs 6.4%). Those with at least one adverse event (AE) were significantly more frequent among those vaccinated with BioNTech than those with CoronaVac (BS: 86.4% vs 45%; FMF: 83.4% vs 53.3%; and RD: 83.3% vs 45.5%). The majority of AEs were mild to moderate and transient and this was true for either vaccine. There were also AEs that required medical attention in all study groups following CoronaVac (BS: 5.5%, FMF: 3.3%, and RD:2.9%) or BioNTech (BS: 5.4%, FMF: 1.9%, and RD: 4.7%). The main causes for medical assistance were disease flare and cardiovascular events. Patients with BS (16.0%) and FMF (17.4%) were found to flare significantly more frequently when compared to those with RD (6.0%) ( p < 0.001). This was true for either vaccine. BS patients reported mainly skin-mucosa lesions; there were however, 11 (4.3%) who developed major organ attack such as uveitis, thrombosis or stroke. Flare in FMF patients were associated mainly with acute serositis with or without fever. Arthralgia/arthritis or inflammatory back pain were observed mainly in the RD group. Our study demonstrates that BS and FMF patients vaccinated with either CoronaVac or BioNTech demonstrated similar AE profile and frequency compared to RD patients. AEs that required physician consultation or hospitalization occurred in all study groups after either CoronaVac or BioNTech. Increased frequency of flares in BS and FMF compared to that seen in RD might reflect defects in innate immunity and deserves further investigation. Caution should be required when monitoring these patients after vaccination.

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Section: Introductionmentioning

confidence: 99%

Safety of SARS-CoV-2 vaccination in patients with Behcet’s syndrome and familial Mediterranean fever: a cross-sectional comparative study on the effects of M-RNA based and inactivated vaccine

et al. 2022

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“…This patient had repeated remissions and relapses of AOSD but was relieved with low dose of steroids and achieved drug-free remission status over the past 2 years before vaccination, and improved symptoms by treating with TCZ for a flare that occurred after vaccination. In another case, AOSD flared after the first dose of ChAdOx1 nCov-19 vaccination while maintaining low disease activity of AOSD with etanercept, and symptoms significantly improved as etanercept was change to TCZ [11].…”

Section: Discussionmentioning

confidence: 99%

“…As of concern, several cases of flares of other AIAIDs [9,35] or new diagnoses of AOSD due to COVID-19 vaccination have been reported [12][13][14][15][16]. On the other hand, to the best of our knowledge, there have been only two cases of flare up of AOSD reported following COVID-19 vaccination [10,11]. There was one case of flare of AOSD after the second dose of COVID-19 mRNA vaccine was reported in Japan [10].…”

Section: Discussionmentioning

confidence: 99%

“…Given the close temporal relationship between vaccination and onset of symptoms despite TCZ preventing the cytokine storm and ruling out other infections and malignancies, we concluded that vaccination against COVID-19 may have triggered the occurrence of flare of AOSD. South Korea [11] The mechanisms underlying AOSD flare-ups after vaccination have not yet been elucidated. A possible pathologic mechanism is that mRNA transcribes into spike glycoproteins after inoculation, and these proteins are displayed on antigen-presenting cells by molecular mimicry, leading to an acute immune response [37].…”

Section: Discussionmentioning

confidence: 99%

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

et al. 2022

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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.

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“…Whether this syndrome is an extremely rare adverse reaction to the vaccine or coincidental is not known. Sporadic cases of AOSD flares after ChAdOx1 vaccine [ 42 , 43 ] and the development of MAS after BNT162b2 vaccine [ 44 ] administration have been described. Another report found that the incidence of adverse events was similar between patients with rheumatic diseases and healthy controls, with a low incidence of flare (<5%) among the former [ 45 ].…”

Section: Discussionmentioning

confidence: 99%

Adult-Onset Still’s Disease-like Syndrome following COVID-19 Vaccination: A Case Report and Review of the Literature

et al. 2022

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Adult-onset Still’s disease (AOSD)-like syndrome has rarely been reported as a complication of COVID-19 vaccination. This study reports a 31-year-old female patient who presented with fever, myalgia, arthralgia, pleuropericarditis, leukocytosis, and transaminitis following ChAdOx1 vaccination, and met Yamaguchi’s criteria. A PubMed literature search, performed up until March 2022, identified 10 such cases. A total of 11 cases, including the one in this report, developed AOSD-like syndrome after administration of the viral vector (ChAdOx1) vaccine (six patients) and mRNA vaccine (five patients: BNT162b2 in four and mRNA-1273 in one). There were four male and seven female patients, with their median (Q1, Q3) age and the onset of symptoms after vaccination being 36 years (29, 45) and 10 days (6, 13), respectively. Fever (100%), arthralgia/arthritis (90.9%), skin rashes (81.8%), and sore throat (81.8%) were the main clinical findings. Pericarditis (45.5%), myocarditis/cardiac dysfunction (36.4%), pleuritis (54.6%), and pulmonary infiltrations (36.4%) were also common. One patient developed macrophage activation syndrome. One patient responded well to non-steroidal anti-inflammatory drugs, and the other six showed a good response to high-dose corticosteroids alone. Of the remaining four patients, who showed partial responses to high dose corticosteroids, showed good responses to biological agents. AOSD-like syndrome following COVID-19 vaccination shared many similar clinical features and treatment outcomes to those of idiopathic AOSD (but with a higher prevalence of cardiopulmonary involvement in the former). Physicians should be aware of this extremely rare complication to achieve early diagnosis and provide proper management.

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A flare of Still’s disease following COVID-19 vaccination in a 34-year-old patient

Cited by 19 publications

References 26 publications

Safety of SARS-CoV-2 vaccination in patients with Behcet’s syndrome and familial Mediterranean fever: a cross-sectional comparative study on the effects of M-RNA based and inactivated vaccine

Safety of SARS-CoV-2 vaccination in patients with Behcet’s syndrome and familial Mediterranean fever: a cross-sectional comparative study on the effects of M-RNA based and inactivated vaccine

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

Adult-Onset Still’s Disease-like Syndrome following COVID-19 Vaccination: A Case Report and Review of the Literature

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