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; Masahito Yamada

doi:10.2169/internalmedicine.8550-21

This article has now been updated. Please use the final version.

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, Masahito Yamada

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Keywords: IgG4-related disease, hypertrophic pachymeningitis, myeloperoxidase anti-neutrophil cytoplasmic antibody, granulomatosis with polyangiitis, dura mater biopsy, granulomatous inflammation

JOURNAL OPEN ACCESS Advance online publication

Article ID: 8550-21

DOI https://doi.org/10.2169/internalmedicine.8550-21

The final version of this article is now available: Vol. 61 (2022), No. 12 pp. 1903-1906

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Abstract

We 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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