O42. SEVERE PYREXIAL ILLNESS FOLLOWING PNEUMOCOCCAL VACCINATION IN BEHÇET’S DISEASE

Abstract

Background: We report the case of a 44-year-old man with Behçet's disease who developed a severe pyrexial illness after pneumococcal and influenza vaccination. On the evening of vaccination, he developed pain in his right upper arm where he had the pneumococcal polysaccharide vaccine. The following day he became systemically unwell with fever, rigors, vomiting and diarrhoea. His condition deteriorated the subsequent day. On arrival at hospital, he was found to be hypotensive, tachycardic and pyrexial. He had a diffusely swollen, erythematous and tender right upper arm. Blood tests revealed stage 3 acute kidney injury, CRP of 500 and serum lactate of 7. Methods: He remained hypotensive despite fluid resuscitation and was transferred to intensive care unit for blood pressure support. He was commenced on intravenous antibiotics and intravenous steroids. Blood cultures were negative and chest X-ray showed no consolidation. Ultrasound scan of the right upper arm showed no organized collection. He made a swift recovery with normalization of serum creatinine within 3 days. He was eventually discharged home on prednisolone 30mg. Results: This gentleman's diagnosis of Behçet's disease was confirmed by the Birmingham National Centre of Excellence for Behçet's Syndrome. He was on methotrexate and prednisolone 14mg. He has a history of erythema nodosum, mouth ulcers since childhood, several years' history of genital ulceration and recurrent uveitis. He also reported severe pathergy-like reactions, including exaggerated skin reaction to venepuncture and, notably, fever after tattooing. There is no known Turkish ancestry. There were no other reported unusual reactions to pneumococcal or influenza vaccination at the same GP practice. It is unclear whether this episode of pyrexial illness represents an infection, an exaggerated immune response or both. The negative blood culture and lack of abscess on ultrasound scan suggest that an immune response is likely to be a potent driver of this severe inflammatory syndrome. Conclusion: Severe systemic reactions following pneumococcal vaccination have been reported in Cryopyrin-associated periodic syndromes and Behçet's disease. Four Behçet's patients who developed severe inflammation after pneumococcal vaccination, three of whom were of Turkish origin, have been reported. Fever, systemic upset and elevated CRP were reported in three patients. However, to the authors' knowledge, such significant hypotension and acute kidney injury have not been reported in the literature. The pathogenesis of this inflammatory syndrome remains unclear and further study is warranted.