Adult‐onset Still disease post‐adenovirus vector <scp>COVID</scp>‐19 vaccine

Abstract

There are emerging cases of immune-mediated diseases post COVID-19 vaccination.1 We report a case of adult-onset Still disease (AOSD) after adenoviral vector vaccination (AstraZeneca). There has been one other case in the literature of documented AOSD post-adenoviral vector vaccination; the present case lends weight to a possible rare association. Mr NS, a 53-year-old marathon runner with no significant past medical history, presented to his general practitioner with a 1-week history of fatigue post initial vaccination with ChAdOx1 nCoV-19/AZD1222 (AstraZeneca). Workup at that stage was unremarkable. Ten weeks following his vaccination, he was urgently referred to the Emergency Department on 22 July 2021 with headache, arthralgia, malaise and fatigue. He was also noted to be thrombocytopenic with a platelet count of 88 × 109/L (reference range 150–400) associated with a raised D-dimer of 2.36 μg/mL FEU (reference range 0.00–0.40), and there was concern regarding the risk of thrombosis with thrombocytopenia. He complained of a 2-week history of worsening headaches, arthralgia, pharyngitis, rash and drenching sweats. Examination revealed, quotidian fevers of >39°C and a salmon-coloured rash over the anterior chest, back and limbs (Fig. 1). Mr NS was extremely stiff with pain on movement of the knees, in particular the left side, and no joint effusion was noted. Mild weakness was noted at the hip and shoulder girdles with difficulty sustaining muscle resistance. No lymphadenopathy was noted. Investigations carried out in the community 2 days prior to admission revealed: C-reactive protein (CRP) 56 mg/L (reference range <5), ferritin 3140 μg/L (reference range 30–300), erythrocyte sedimentation rate (ESR) 17 mm (reference range 1–15), aspartate aminotransferase 68 U/L (reference range 10–40) and alanine transaminase 62 U/L (reference range 5–40). During admission, levels of ferritin, CRP and ESR peaked at 10200 μg/L, 237 mg/L and 85 mm/h, respectively. In the setting of thrombocytopenia with raised D-dimer, a computed tomography pulmonary angiogram scan was performed that ruled out pulmonary embolism; no pulmonary infiltrate was noted. Abdominal ultrasound confirmed mild splenomegaly without lymphadenopathy. Autoimmune screening including antinuclear antibody, antineutrophil cytoplasmic antibody, extractable nuclear antigen, double-stranded DNA and complement studies were normal. A diagnosis of AOSD by Yamaguchi criteria was made.2 Notably, these criteria are not considered diagnostic of AOSD, and this remains a diagnosis of exclusion, therefore infectious and malignant aetiology were considered and excluded up to and including the positron emission tomography scan, which was unremarkable. Oral prednisolone 60 mg was initiated with remarkable improvement in joint symptoms and resolution of daily temperatures. Three days following the initiation of steroid therapy, the patient was discharged home well on oral prednisolone. At follow up 2 weeks later, Mr NS was asymptomatic from AOSD. Our case highlights a temporal association between AOSD and adenoviral vaccination. The ChAdOx1 nCoV-19 vaccine has been linked to three reported cases of haemophagocytic lymphohistiocytosis, a clinical syndrome not dissimilar to the macrocyte activation syndrome associated with AOSD. In these cases, symptoms developed 10, 7 and 8 days following the first dose of the vaccine.3 Interestingly, a case of AOSD following vaccination with mRNA-1273 COVID-19 vaccine (Moderna) has also been reported.4 In that case, the patient developed symptoms 5 days following the second dose of the vaccine, approximately 1 month after the initial dose. Fortunately, the above-described effects appear to be exceedingly rare, given that over 5 billion people worldwide have received COVID-19 vaccination in some form. Further information on the pathophysiological effects of various COVID-19 vaccines is required to provide further guidance in this public health issue. No conflict of interest is declared. The patient has provided consent for publication of the above article.

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