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

被引：4

作者：

Mori, Makoto ^{[1
]}

Sakai, Kenji ^{[2
]}

Saito, Katsuhiko ^{[3
]}

Nojima, Takayuki ^{[4
]}

Mohri, Masanao ^{[5
]}

Matsubara, Keitaro ^{[1
]}

Hayashi, Shigeru ^{[1
]}

Yamada, Masahito ^{[2
]}

机构：

[1] Toyama City Hosp, Dept Neurol, Toyama, Japan

[2] Kanazawa Univ Grad Sch Med Sci, Dept Neurol & Neurobiol Aging, Kanazawa, Ishikawa, Japan

[3] Toyama City Hosp, Dept Diagnost Pathol, Toyama, Japan

[4] Kanazawa Univ Hosp, Dept Diagnost Pathol, Kanazawa, Ishikawa, Japan

[5] Toyama City Hosp, Dept Neurosurg, Toyama, Japan

来源：

INTERNAL MEDICINE | 2022年 / 61卷 / 12期

关键词：

IgG4-related disease; hypertrophic pachymeningitis; myeloperoxidase anti-neutrophil cytoplasmic antibody; granulomatosis with polyangiitis; dura mater biopsy; granulomatous inflammation; DISEASE;

D O I：

10.2169/internalmedicine.8550-21

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

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 IgG4positive 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.

引用

页码：1903 / 1906

页数：4

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