Noninfectious Ascending Aortitis: A Case Series of 64 Patients
Noninfectious Ascending Aortitis: A Case Series of 64 Patients
复制标题
DOI：
10.3899/jrheum.090081
复制
发表时间：
2009-10-01
影响因子：
3.9
通讯作者：
Warrington, Kenneth J.
中科院分区：
文献类型：
--
作者：
Liang, Kimberly P.;Chowdhary, Vaidehi R.;Warrington, Kenneth J.
Objective.明确非感染性上行性结肠炎的临床表现和组织病理学特征。对2000年1月1日至2006年2月28日期间在马约诊所接受升主动脉瘤切除术的有活动性非感染性乳腺炎组织学证据的患者进行回顾性病历和组织病理学审查。记录临床病理特征，包括人口统计学、临床表现、实验室检查、影像学检查、组织病理学、并发症、治疗和结局。确定了64例患者（50%为女性）;大多数为白人（83%）和老年人（平均年龄69.1岁）。在最初的表现，45%的患者有神经系统相关症状，33%无症状，12.5%有全身症状，4.7%有颅动脉症状，9.4%有风湿性多肌痛（PMR）症状。大多数（81%）是“孤立的”变异，没有风湿病史。平均术前红细胞沉降率为16.2 ± 23.3 mm/h（n = 20）。72%的患者存在其他血管成像异常，包括主动脉分支狭窄和/或扩张以及胸或腹降动脉瘤。巨细胞占71.9%。中位随访时间为15.4个月，6例（9.4%）患者死亡。仅22例（34%）患者接受皮质类固醇治疗，对复发动脉瘤、破裂或夹层的影响尚不确定。即使没有巨细胞动脉炎（GCA）或PMR的病史、症状或体征，也经常发生非感染性上行性动脉炎。一旦发现，应密切跟踪此类患者，因为大多数患者有其他血管异常。需要更多的研究来确定上行性乳腺炎的监测、检测和治疗的最佳策略，上行性乳腺炎可能代表不同于经典GCA的临床实体。（2009年8月1日首次发布; J Rheumol 2009;36：2290-7; doi：10.3899/jrheum.090081）
Objective. To identify the clinical presentation and histopathologic characteristics of noninfectious ascending aortitis.Methods. A retrospective medical record and histopathology review was performed of patients with histologic evidence of active noninfectious aortitis who underwent ascending aortic aneurysm resection at Mayo Clinic between January 1, 2000, and February 28, 2006. Clinicopathologic features were recorded, including demographics, clinical presentation, laboratory, imaging findings, histopathology, complications, treatment, and outcome.Results. Sixty-four patients (50% women) were identified; the majority were Caucasian (83%) and elderly (mean age 69.1 yrs). Upon initial presentation, 45% had aneurysm-related symptoms, 33% were asymptomatic, 12.5% had constitutional symptoms, 4.7% had symptoms referable to cranial arteries, and 9.4% had polymyalgia rheumatica (PMR) symptoms. The majority (81%) were of "isolated" variant, with no rheumatologic history. Mean preoperative erythrocyte sedimentation rate was 16.2 +/- 23.3 mm/h (n = 20). Additional vascular imaging abnormalities were present in 72% of patients, including stenoses and/or ectasia of major aortic branches and descending thoracic or abdominal aneurysms. Giant cells were seen in 71.9%. Median followup time was 15.4 months, during which 6 (9.4%) patients died. Only 22 (34%) patients received corticosteroids, with uncertain effect on development of recurrent aneurysms, rupture, or dissections.Conclusion. Noninfectious ascending aortitis frequently occurs even in the absence of history, symptoms, or signs of giant cell arteritis (GCA) or PMR. When discovered, such patients should be followed closely, as a majority have additional vascular abnormalities. More studies are needed to determine optimal strategies for surveillance, detection, and treatment of ascending aortitis, which may represent a clinical entity distinct from classical GCA. (First Release Aug 1 2009; J Rheumatol 2009;36:2290-7; doi: 10.3899/jrheum.090081)