Tanboon Jantima, Felicella Michelle Madden, Bilbao Juan, Mainprize Todd, Perry Arie
Department of Pathology, Faculty of Medicine, Siriraj Hospital, Mahidol University, Bangkok.
Clin Neuropathol. 2013 Jul-Aug;32(4):291-7. doi: 10.5414/NP300575.
IgG4-related disease (IgG4-RD) is a recently recognized fibro-inflammatory condition which often shows a dramatic response to steroid therapy. IgG4-RD can present either as a single lesion or as a systemic multi-organ disorder. Common histological findings include a dense lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells, storiform fibrosis, and phlebitis. Although diagnostic criteria for IgG4-RD have been proposed in many organs/sites, they are not well established in the central nervous system. Published data on IgG4-RD in meninges is also limited. To our knowledge, only 15 potential cases of meningeal IgG4-RD have been reported. We add a case of probable IgG4-related pachymeningitis in a 42-year-old woman who presented with headache and left transverse sinus obstruction. Follow-up after 2-months of high dose steroids shows dramatic clinical and imaging improvement. The differential diagnosis for IgG4-related pachymeningitis, including lymphoplasmacyte-rich meningioma, idiopathic hypertrophic pachymeningitis, and lymphoproliferative disease is discussed.
IgG4相关性疾病（IgG4-RD）是一种最近才被认识的纤维炎症性疾病，通常对类固醇治疗有显著反应。IgG4-RD可表现为单个病变或全身性多器官疾病。常见的组织学表现包括富含IgG4阳性浆细胞的致密淋巴浆细胞浸润、束状纤维化和静脉炎。尽管已经提出了IgG4-RD在许多器官/部位的诊断标准，但在中枢神经系统中尚未完全确立。关于脑膜IgG4-RD的已发表数据也很有限。据我们所知，仅报告了15例可能的脑膜IgG4-RD病例。我们补充了一例42岁女性可能的IgG4相关性硬脑膜炎病例，该患者表现为头痛和左侧横窦阻塞。高剂量类固醇治疗2个月后的随访显示临床和影像学有显著改善。本文讨论了IgG4相关性硬脑膜炎的鉴别诊断，包括富含淋巴浆细胞的脑膜瘤、特发性肥厚性硬脑膜炎和淋巴增殖性疾病。
