A new clinicopathological entity of IgG4-related inflammatory abdominal aortic aneurysm
A new clinicopathological entity of IgG4-related inflammatory abdominal aortic aneurysm
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DOI：
10.1016/j.jvs.2008.11.072
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发表时间：
2009-05-01
影响因子：
4.3
通讯作者：
Kasashima, Fuminori
中科院分区：
文献类型：
--
作者：
Kasashima, Satomi;Zen, Yoh;Kasashima, Fuminori
目的：近年来，免疫球蛋白（Ig）G4与特发性硬化病变的关系备受关注。igg4相关疾病最初被描述为胰腺（自身免疫性胰腺炎），并已扩展到各种器官系统。我们之前报道过炎症性腹主动脉瘤（IAAA）可能是igg4相关疾病的表现之一。在本研究中，我们试图阐明与igg4相关的IAAA的临床特征。方法：本研究包括23例IAAA和40例动脉粥样硬化性腹主动脉瘤（AAA）。检查了临床表现、实验室结果和病理特征。13例动脉瘤在组织学上符合igg4相关的iaaa。这些病例占所有外科AAAs的5%，占iaas的57%。与非igg4相关的IAAA相比，igg4相关病例的特征是较少伴有腹部或背部疼痛。IgG4相关病例血清IgG4浓度显著升高。有趣的是，与igg4相关的LAAA患者往往表现为过敏体质，如药物过敏、自身免疫性疾病、血清IgE浓度高、抗核抗体滴度高。病理上，igg4相关病例的特点是外膜增厚更明显，igg4阳性浆细胞浸润更多。3例非igg4相关的病例在首次出现时出现动脉瘤破裂，而没有igg4相关的病例出现破裂。结论：认识igg4相关IAAA的一种新的疾病实体似乎很重要，因为它在临床和病理上不同于传统的aAAA和非igg4相关的IAAA。（中华花瓶外科杂志2009；49:1264-71）
Objective: Recently, the relationship between immunoglobulin (Ig)G4 and idiopathic sclerosing lesions has attracted much attention. IgG4-related disease was first described with regard to the pancreas (autoimmune pancreatitis), and has been expanded to various organ systems. We previously reported that inflammatory abdominal aortic aneurysm (IAAA) could be one of the manifestations of IgG4-related disease. In this study, we tried to elucidate the clinical characteristics of IgG4-related IAAA.Methods: This study consisted of 23 cases of IAAA and 40 cases of atherosclerotic abdominal aortic aneurysm (AAA). Clinical presentation, laboratory findings, and pathological features were examined. Aneurysms of 13 cases histologically corresponded to IgG4-related IAAA.Results. Those cases accounted for 5% of all surgical AAAs, and 57% of IAAAs. Compared to non-IgG4-related IAAA, IgG4-related cases were characterized by less frequent association with abdominal or back pain. Serum IgG4 concentrations were significantly elevated in IgG4-related cases. Interestingly, patients with IgG4-related LAAA frequently showed an allergic constitution, such as drug allergy, autoimmune diseases, high serum IgE concentrations, and a high titer of antinuclear antibody. Pathologically, IgG4-related cases were characterized by more significant thickening of the adventitia and more numerous IgG4-positive plasma cell infiltrations. Three non-IgG4-related cases showed aneurysmal rupture at the time of first presentation, whereas no IgG4-related cases showed rupture.Conclusion: Recognizing a new disease entity of IgG4-related IAAA seems important because this was clinically and pathologically different from conventional aAAA and non-IgG4-related IAAA. (J Vase Surg 2009;49:1264-71.)