• Internal medicine · Jun 2022

    Case Reports

    Hypertrophic pachymeningitis with characteristics of both IgG4-related disorders and granulomatosis with polyangiitis.

    • Makoto Mori, Kenji Sakai, Katsuhiko Saito, Takayuki Nojima, Masanao Mohri, Keitaro Matsubara, Shigeru Hayashi, and Masahito Yamada.
    • Department of Neurology, Toyama City Hospital, Japan.
    • Intern. Med. 2022 Jun 15; 61 (12): 1903-1906.

    AbstractWe herein report a 73-year-old man with isolated hypertrophic pachymeningitis (HP) showing serological and pathological characteristics of both IgG4-related disorders and granulomatosis with polyangiitis. The patient presented with chronic onset headaches and ophthalmalgia. Brain magnetic resonance imaging (MRI) revealed a hypertrophic enhanced dura mater. Serum IgG4 and myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA) levels were elevated. A dura mater biopsy showed infiltration of numerous IgG4-positive plasma cells and granulomatous inflammation without apparent vasculitic lesions, storiform fibrosis, or obstructive phlebitis. Corticosteroid treatments improved his clinical symptoms and MRI findings. There have been reports of MPO-ANCA-positive IgG4-related HP presenting as granulomatous inflammation in the dura mater.

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