未经证实:肥厚性硬脑膜炎(HP)是一种罕见的炎症性疾病,其特征是颅内或脊髓硬脑膜局部或弥漫性增厚。HP最常见的原因是抗中性粒细胞胞浆抗体(ANCA),其次是IgG4。然而,报道了少数IgG4-HP共表达ANCA的病例.在这里,我们介绍了3例IgG4-HP共表达ANCA的病例,并回顾了相关文献,以记录这两种HP病因的重叠是一种潜在的临床模式.
UNASSIGNED:我们回顾性分析了我们中心的3例IgG4-HP共表达ANCA的患者,并查阅了PubMed数据库,以查找1976年至2022年4月以英文报道的其他相关病例。我们用了以下关键词:硬脑膜炎,脑膜炎,dura,抗中性粒细胞胞浆抗体,髓过氧化物酶,和蛋白酶-3.我们分析了临床,血清学,放射学,根据ACR和ChapelHill标准以及ANCA相关血管炎(AAV)的指数移动平均(EMA)算法和IgG4-RD综合诊断标准,获得的病例的病理特征。
UNASSIGNED:我们共分析了10例病例:7例文献报道和3例患者(52岁和61岁的女性和65岁的男性)。IgG4相关疾病(IgG4-RD)的诊断是确定的4例,在三种情况下是可能的。8例患者有ANCA抗髓过氧化物酶(MPO),和两个具有抗蛋白酶-3(PR3)的ANCA。两名患者同时患有IgG4-RD和AAV,而其他人只有ANCA血清阳性,没有其他AAV的临床或病理标志物。
未经评估:关于HP,我们再次确认存在IgG4-RD和AAV重叠综合征.同时,我们的综述不支持IgG4-RD的ANCA阳性起因于B细胞过度反应的假设.我们推测IgG4-RD和AAV具有相似或相关的发病机制,尽管发现IgG4和ANCA在这些病理生理过程中的作用还需要进一步研究.
UNASSIGNED: Hypertrophic pachymeningitis (HP) is a rare inflammatory disorder characterized by local or diffuse thickening of the intracranial or spinal dura mater. The most frequent cause of HP is antineutrophil cytoplasmic antibodies (ANCA), followed by IgG4. However, few cases of
IgG4-HP coexpressing ANCA have been reported. Herein, we present three cases of
IgG4-HP coexpressing ANCA and
review the relevant literature to document the overlap of these two HP causes as a potential clinical pattern.
UNASSIGNED: We retrospectively analyzed three patients with IgG4-HP coexpressing ANCA in our center and consulted the PubMed database to find other relevant cases reported in English from 1976 to April 2022. We used the following keywords: pachymeningitis, meningitis, dura, antineutrophil cytoplasmic antibody, myeloperoxidase, and proteinase-3. We analyzed the clinical, serological, radiological, and pathological characteristics of the obtained cases based on the ACR and Chapel Hill criteria and the exponential moving average (EMA) algorism for ANCA-associated vasculitis (AAV) and the IgG4-RD Comprehensive Diagnostic Criteria.
UNASSIGNED: We analyzed a total of 10 cases: seven literature reports and our three patients (52- and 61-year-old women and a 65-year-old man). The
IgG4-related disease (
IgG4-RD) diagnoses were definitive in four cases, and probable and possible in three cases. Eight patients had ANCA against myeloperoxidase (MPO), and two had ANCA against proteinase-3 (PR3). Two patients had both IgG4-RD and AAV, while the others only had ANCA seropositivity without additional clinical or pathological markers of AAV.
UNASSIGNED: With regard to HP, we reconfirmed the existence of the IgG4-RD and AAV overlap syndrome. Meanwhile, our
review does not support the hypothesis that ANCA positivity in
IgG4-RD results from an excessive B-cell response. We speculate that IgG4-RD and AAV have similar or associated pathogeneses, although uncovering the role of
IgG4 and ANCA in these pathophysiological processes requires further investigation.