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Vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) syndrome presenting as recurrent aseptic peritonitis in a patient receiving peritoneal dialysis: a case report by Fukuda, Natsuki (author);Kanai, Daisuke (author);Hoshino, Kaoru (author);Fukuda, Yuriko (author);Morita, Ryutaro (author);Ishikawa, Yuki (author);Kanaoka, Tomohiko (author);Toya, Yoshiyuki (author);Kirino, Yohei (author);Wakui, Hiromichi (author);Tamura, Kouichi (author) is a Medicine article available to read on EtoBox.

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## Background Vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) syndrome is caused by mutations in the ubiquitin-activating enzyme1 (__UBA1__) gene and characterised by an overlap between autoinflammatory and haematologic disorders. ## Case presentation We reported a case of a 67-year-Japanese man receiving peritoneal dialysis (PD) who had recurrent aseptic peritonitis caused by the VEXAS syndrome. He presented with unexplained fevers, headache, abdominal pain, conjunctival hyperaemia, ocular pain, auricular pain, arthralgia, and inflammatory skin lesions. Laboratory investigations showed high serum C-reactive protein concentration and increased cell count in PD effluent. He was treated with antibiotics for PD-related peritonitis, but this was unsuccessful. Fluorine-18-fluorodeoxyglucose (FDG) positron emission tomography/computed tomography images demonstrated intense FDG uptake in his left superficial temporal artery, nasal septum, and bilateral auricles. The working diagnosis was giant cell arteritis, and he was treated with oral prednisolone (PSL) 15 mg daily with good response. However, he was unable to taper the dose to less than 10 mg daily because his symptom

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Author

Fukuda, Natsuki (author);Kanai, Daisuke (author);Hoshino, Kaoru (author);Fukuda, Yuriko (author);Morita, Ryutaro (author);Ishikawa, Yuki (author);Kanaoka, Tomohiko (author);Toya, Yoshiyuki (author);Kirino, Yohei (author);Wakui, Hiromichi (author);Tamura, Kouichi (author)

Publisher

Springer Science and Business Media LLC

Published

2024

Language

EN

Field

Medicine (Health Sciences)