Pachymeningitis associated with IgG4-related disease and ANCA positivity: Case report and review of the literature.

Félicien Gautier, Lisa Neumann, Homa Adle-Biassete,Emma Rubenstein,Anne-Laure Bernat, Alice Chimon,Stéphane Mouly,Damien Sène,Cloé Comarmond

Autoimmunity reviews（2023）

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摘要

Pachymeningitis with IgG4 and ANCA seems a localized disease to the head and neck. Leptomeningeal biopsy commonly found IgG4 criteria and no vasculitis. All patients responded well to steroid therapy and immunosuppressive drugs, especially rituximab, with clinical and radiological improvement but relapse and/or sequelae are not uncommon.

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ANCA,IgG4-related disease,Pachymeningitis,Vasculitis

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