Hypertrophic Pachymeningitis with Characteristics of Both IgG4-related Disorders and Granulomatosis with Polyangiitis

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...

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Autores principales: Mori, Makoto, Sakai, Kenji, Saito, Katsuhiko, Nojima, Takayuki, Mohri, Masanao, Matsubara, Keitaro, Hayashi, Shigeru, Yamada, Masahito
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The Japanese Society of Internal Medicine 2021
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9259806/
https://www.ncbi.nlm.nih.gov/pubmed/34803104
http://dx.doi.org/10.2169/internalmedicine.8550-21
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author Mori, Makoto
Sakai, Kenji
Saito, Katsuhiko
Nojima, Takayuki
Mohri, Masanao
Matsubara, Keitaro
Hayashi, Shigeru
Yamada, Masahito
author_facet Mori, Makoto
Sakai, Kenji
Saito, Katsuhiko
Nojima, Takayuki
Mohri, Masanao
Matsubara, Keitaro
Hayashi, Shigeru
Yamada, Masahito
author_sort Mori, Makoto
collection PubMed
description 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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spelling pubmed-92598062022-07-19 Hypertrophic Pachymeningitis with Characteristics of Both IgG4-related Disorders and Granulomatosis with Polyangiitis Mori, Makoto Sakai, Kenji Saito, Katsuhiko Nojima, Takayuki Mohri, Masanao Matsubara, Keitaro Hayashi, Shigeru Yamada, Masahito Intern Med Case Report 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. The Japanese Society of Internal Medicine 2021-11-20 2022-06-15 /pmc/articles/PMC9259806/ /pubmed/34803104 http://dx.doi.org/10.2169/internalmedicine.8550-21 Text en Copyright © 2022 by The Japanese Society of Internal Medicine https://creativecommons.org/licenses/by-nc-nd/4.0/The Internal Medicine is an Open Access journal distributed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License. To view the details of this license, please visit (https://creativecommons.org/licenses/by-nc-nd/4.0/).
spellingShingle Case Report
Mori, Makoto
Sakai, Kenji
Saito, Katsuhiko
Nojima, Takayuki
Mohri, Masanao
Matsubara, Keitaro
Hayashi, Shigeru
Yamada, Masahito
Hypertrophic Pachymeningitis with Characteristics of Both IgG4-related Disorders and Granulomatosis with Polyangiitis
title Hypertrophic Pachymeningitis with Characteristics of Both IgG4-related Disorders and Granulomatosis with Polyangiitis
title_full Hypertrophic Pachymeningitis with Characteristics of Both IgG4-related Disorders and Granulomatosis with Polyangiitis
title_fullStr Hypertrophic Pachymeningitis with Characteristics of Both IgG4-related Disorders and Granulomatosis with Polyangiitis
title_full_unstemmed Hypertrophic Pachymeningitis with Characteristics of Both IgG4-related Disorders and Granulomatosis with Polyangiitis
title_short Hypertrophic Pachymeningitis with Characteristics of Both IgG4-related Disorders and Granulomatosis with Polyangiitis
title_sort hypertrophic pachymeningitis with characteristics of both igg4-related disorders and granulomatosis with polyangiitis
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9259806/
https://www.ncbi.nlm.nih.gov/pubmed/34803104
http://dx.doi.org/10.2169/internalmedicine.8550-21
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