Division of Rheumatology, Department of Internal Medicine, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea.
Int J Rheum Dis. 2014 May;17(4):420-4. doi: 10.1111/1756-185X.12279. Epub 2014 Jan 22.
To identify the frequency of immunoglobulin G4 (IgG4)-related aortitis in patients who undergo aorta surgery and are diagnosed by pathology as having chronic aortic inflammation and to compare IgG4-related aortitis with other non-infectious aortitises in terms of clinical characteristics.
The aorta specimen pathological reports of 1418 patients who underwent aortic aneurysm or dissection surgery were reviewed. In total, 41 had chronic aortic inflammation without atherosclerosis, cancer or infection. Their aorta biopsy specimens were subjected to IgG4 immunostaining. IgG4-related aortitis was diagnosed if the IgG4-positive plasma cell count exceeded 50 per high power field (HPF), the ratio of IgG4-positive to IgG-positive plasma cells exceeded 50% and dense lymphoplasmacytic infiltration, fibrosis and/or obliterative phlebitis were observed.
Of the 41 non-infectious aortitis cases, 29, six and six had idiopathic aortitis, Takayasu's arteritis and Behcet's aortitis, respectively. Of the 29 idiopathic aortitis cases, three had IgG4-related aortitis. All were male and > 65 years of age. Two had thoracic aortic aneurysms and one had an abdominal aortic aneurysm. Their IgG4-positive plasma cell counts were 60/HPF or higher; lymphoplasmacytic infiltration and/or fibrosis, but not obliterative phlebitis, were observed. The IgG4-related aortitis cases were older (67 [range, 65-69] years) than the Takayasu's arteritis (47.5 [38-58] years) or Behcet's aortitis (47 [31-56] years) cases and more likely to be male than the Takayasu's arteritis cases (100% vs. 17%).
In patients with chronic aortic inflammation, 7% had IgG4-related aortitis. This disease may be more common in older male patients than in other demographic groups.
确定行主动脉手术并经病理诊断为慢性主动脉炎症的患者中 IgG4 相关性大动脉炎的频率，并比较 IgG4 相关性大动脉炎与其他非感染性大动脉炎的临床特征。
回顾了 1418 例行主动脉瘤或夹层手术患者的主动脉标本病理报告。共有 41 例患者无动脉粥样硬化、癌症或感染，但有慢性主动脉炎症。对这些患者的主动脉活检标本进行 IgG4 免疫染色。如果 IgG4 阳性浆细胞计数超过 50 个高倍视野（HPF）、IgG4 阳性浆细胞与 IgG 阳性浆细胞的比例超过 50%，且观察到致密的淋巴浆细胞浸润、纤维化和/或闭塞性静脉炎，则诊断为 IgG4 相关性大动脉炎。
在 41 例非感染性大动脉炎病例中，特发性大动脉炎 29 例，Takayasu 动脉炎 6 例，Behcet 大动脉炎 6 例。在 29 例特发性大动脉炎病例中，有 3 例为 IgG4 相关性大动脉炎。这 3 例均为男性，年龄均>65 岁。2 例为胸主动脉瘤，1 例为腹主动脉瘤。这 3 例患者的 IgG4 阳性浆细胞计数均为 60/HPF 或更高；观察到淋巴浆细胞浸润和/或纤维化，但未观察到闭塞性静脉炎。IgG4 相关性大动脉炎患者的年龄（67 [范围：65-69] 岁）大于 Takayasu 动脉炎（47.5 [38-58] 岁）或 Behcet 大动脉炎（47 [31-56] 岁）患者，且男性比例大于 Takayasu 动脉炎患者（100% vs. 17%）。
在慢性主动脉炎症患者中，7%患有 IgG4 相关性大动脉炎。这种疾病可能在老年男性患者中比其他人群更为常见。
