Division of Rheumatology, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, South Korea.
Division of Cardiology, Department of Medicine, Heart Vascular Stroke Institute, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, South Korea.
Int J Rheum Dis. 2017 Nov;20(11):1751-1762. doi: 10.1111/1756-185X.13194. Epub 2017 Nov 3.
Chronic periaortitis (CP) is a disease characterized by a fibro-inflammatory periaortic cuff and adventitia-predominant fibrosis. CP encompasses idiopathic retroperitoneal fibrosis and inflammatory abdominal aortic aneurysm (AAA), and recent studies have documented overlap between CP and immunoglobulin G4-related disease (IgG4-RD). This study aimed to investigate clinical characteristics and treatment outcomes of patients with CP.
CP patients were identified by retrospective review of 1245 patients with International Classification of Diseases 10th edition code of aortitis or aortic disease. Patients were further classified into IgG4-related and non-IgG4-related CP according to the criteria proposed by a Japanese study.
A total of 61 CP patients were identified. Patients showed a male predominance (70%) with median age of 61 at diagnosis. The abdominal aorta was most commonly involved (84%), while the thoracic aorta was affected in 46% of patients. Twenty-three (38%) patients had accompanying aortic aneurysm. Approximately 60% of patients achieved remission without further relapse during the course. Ten patients were classified as IgG4-related and 25 as non-IgG4-related. There was no significant difference in clinical features and outcomes between groups, with the exception of older age and greater pancreas involvement in IgG4-related patients.
We documented 61 CP patients including 10 IgG4-related cases. CP involved the abdominal aorta in most patients and the thoracic aorta in approximately 50% of patients. IgG4-related CP patients were older and had greater pancreas involvement, but disease outcomes appeared to be similar between IgG4-related and non-IgG4-related CP.
慢性大动脉炎（CP）是一种以纤维炎症性主动脉外膜和外膜为主的纤维化为特征的疾病。CP 包括特发性腹膜后纤维化和炎症性腹主动脉瘤（AAA），最近的研究表明 CP 与 IgG4 相关疾病（IgG4-RD）之间存在重叠。本研究旨在探讨 CP 患者的临床特征和治疗结果。
通过回顾性分析国际疾病分类第 10 版编码为大动脉炎或主动脉疾病的 1245 例患者，确定 CP 患者。根据日本研究提出的标准，将患者进一步分为 IgG4 相关和非 IgG4 相关 CP。
共确定了 61 例 CP 患者。患者以男性为主（70%），诊断时的中位年龄为 61 岁。最常受累的是腹主动脉（84%），而胸主动脉受累的患者占 46%。23 例（38%）患者伴有主动脉瘤。在病程中，约 60%的患者无进一步复发而达到缓解。10 例患者被归类为 IgG4 相关，25 例为非 IgG4 相关。除了 IgG4 相关患者年龄较大且胰腺受累更多外，两组患者的临床特征和结局无显著差异。
我们记录了 61 例 CP 患者，包括 10 例 IgG4 相关病例。CP 最常累及腹主动脉，约 50%的患者累及胸主动脉。IgG4 相关 CP 患者年龄较大且胰腺受累更多，但 IgG4 相关和非 IgG4 相关 CP 患者的疾病结局似乎相似。
