Outcomes Among 196 Patients With Noninfectious Proximal Aortitis

Abstract

Objective. Noninfectious aortitis may occur in the context of a recognized systemic disease or as a topographically limited lesion without systemic features, which is called clinically isolated aortitis (CIA). This study was undertaken to better define and stress the limitations of this diagnostic category in a large population of patients in a single center dedicated to aortic diseases and to suggest recommendations for care.Methods. Records of patients undergoing thoracic aortic surgery (1996)(1997)(1998)(1999)(2000)(2001)(2002)(2003)(2004)(2005)(2006)(2007)(2008)(2009)(2010)(2011)(2012) at the Cleveland Clinic were reviewed to identify 196 patients with histopathologically proven aortitis. Clinical diagnoses (giant cell arteritis [GCA], Takayasu arteritis [TAK], CIA, or Other) were determined at the time of surgery. Clinical features, laboratory findings, and imaging results were recorded throughout the follow-up period. At least 6 months of follow-up data were available for 73 CIA patients.Results. The mean age of the patients at time of surgery was 65.6 years (range 15-88 years); 67% of patients were female, and 90.3% were white. At the time of surgery, 129 patients (65.8%) met criteria for CIA, 42 (21.4%) for GCA, 14 (7.1%) for TAK, and 11 (5.6%) met criteria for other systemic inflammatory diseases. During a mean followup period of 56.2 months, 19% of CIA patients developed new symptoms, 45% developed new radiographic vascular lesions, 40% underwent additional vascular surgery, and 12% died (n = 9). Eleven of 73 patients (15%) initially classified as having CIA developed features of a systemic disease, most often GCA.Conclusion. The majority of patients (66%) with histopathologically proven aortitis have CIA at the time of surgery. CIA patients infrequently report new symptoms over time, but new vascular lesions requiring surgery commonly occur. Serial follow-up including large vessel imaging is strongly advised for all aortitis patients.

2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Giant Cell Arteritis and Takayasu Arteritis

“…Because patients can develop collateral blood vessels to improve distal blood flow, immunosuppressive therapy is recommended as initial therapy in patients with GCA and worsening limb/organ ischemia. However, clinical situations that would warrant consideration of immediate surgical intervention include aortic aneurysms at high risk for rupture and impending/progressive tissue or organ infarction or damage (68)(69)(70).…”

“…The optimal frequency and length of monitoring are not well established and depend on factors including the duration of remission, site of involvement, risk of disease progression, whether the patient is receiving immunosuppressive therapy, and reliability of the patient to report new signs or symptoms (48,69). Clinical monitoring may include history taking, examinations, and laboratory and imaging studies.…”

Microbiomes of Inflammatory Thoracic Aortic Aneurysms Due to Giant Cell Arteritis and Clinically Isolated Aortitis Differ From Those of Non-Inflammatory Aneurysms

“…During a mean follow-up of over 4.5 years, 19% of patients with CIA developed new symptoms, 45% developed new radiographic vascular lesions, 40% underwent additional vascular surgery, and 12% died. Fifteen percent of patients initially classified with CIA developed features of a systemic disease, most often GCA [7]. Among numerous questions raised by such studies are the following: First, is CIA a limited presentation of diseases within a spectrum of LVV, including GCA?…”

Surgery for autoimmune aortitis: unanswered questions

“…The preoperative diagnosis of vasculitis is essential to guide the management of the distal aorta. Disease progression was not evaluated in large and several series; however, the largest series up to date by Clifford and Arafat and their colleagues found that 29% of the patients required distal aortic intervention during an 8year follow-up and many patients developed other vascular lesions [13,29]. Wang and associates found 7 of 15 patients with GCA who had ascending aortic surgery developed the distal aortic disease, and 5 required distal reinterventions in a mean time of 58 months.…”

“…Aortic diameters at the level of the mid arch and the maximum descending aortic diameters were decreased post-stenting. Although the decrease in diameters was not statistically significant, the stent graft succeeded to stop the progressive aortic expansion in patients with aortitis [1,13,29]. Patients with active inflammation of the aortic wall may benefit from endovascular interventions since open repair is associated with the formation of pseudoaneurysm at the suture line [35,36].…”