Cases Of Aortitis Research Articles

Clinical and pathological spectrum of aortitis in a Chinese cohort

BackgroundThis study aimed to explore the clinical and pathological features of aortitis in China, which is a rare disease that is often overlooked preoperatively. MethodsWe reviewed the records of 2950 patients who underwent aortic surgery at Wuhan Asia General Hospital from 2016 to 2023. Clinical and pathological data were collected and compared across different groups. ResultsOut of 2950 patients, 15 had healed aortitis, 2 were healed Takayasu aortitis (TAK), and 13 were not further classified. Forty-two had active aortitis, including clinically isolated aortitis ([CIA], 42.9%), infectious aortitis ([IA], 26.2%), TAK (16.7%), and Behçet's syndrome ([BS], 14.3%), half of these cases were not recognized preoperatively. All patients who developed perivalvular leakage during follow-up had concurrent non-infectious valvulitis with mixed inflammatory pattern at the time of initial surgery. Seventeen out of 18 patients with CIA survived without complications, as did 8 out of 11 patients with IA, 6 out of 7 patients with TAK, and 2 out of 6 patients with BS. ConclusionsHalf of the aortitis cases were initially diagnosed by pathologists. Noninfectious valvulitis with mixed inflammatory pattern is a risk factor for perivalvular leakage. BS is associated with a higher rate of complications. Patients with CIA have a good prognosis in China, which is different from the West.

Post-operative outcomes of inflammatory thoracic aortitis: a study of 41 patients from a cohort of 1119 surgical cases.

Aortitis is found in 2-12% of thoracic aortic aneurysm repair/replacement surgeries. Yet little is known about such patients' post-operative outcomes or the role of post-operative corticosteroids. The study was undertaken across three tertiary referral hospitals in Sydney, Australia. Prospectively collected data for all thoracic aortic repair/replacement patients between 2004 and 2018 was accessed from a national surgical registry and analysed. Histopathology records identified cases of inflammatory aortitis which were subclassified as clinically isolated aortitis (CIA), giant cell arteritis (GCA), Takayasu (TAK) or other aortitis. Between-group outcomes were compared utilising logistic and median regression analyses. Between 2004 and 2018, a total of 1119 thoracic aortic surgeries were performed of which 41 (3.7%) were inflammatory aortitis cases (66% CIA, 27% GCA, 5% TAK, 2% other). Eight out of 41 (20%) aortitis patients received post-operative corticosteroids. Compared to non-aortitis patients, the aortitis group was predominantly female (53.7% vs. 28.1%, p < 0.01), was older (mean 70 vs. 62years, p < 0.01) and had higher prevalence of hypertension (82.9% vs. 67.1%, p = 0.03) and pre-operative immunosuppression (9.8% vs. 1.4%, p < 0.01). There was no difference (p > 0.05) between aortitis and non-aortitis groups for 30-day mortality (7.3% vs 6.5%), significant morbidity (14.6% vs. 22.4%), or infection (9.8% vs. 6.4%). Outcomes were similar for the non-corticosteroid-treated aortitis subgroup. Histologic evidence of inflammatory thoracic aortitis following surgery did not affect post-operative mortality or morbidity. Withholding corticosteroids did not adversely affect patient outcomes. These findings will assist rheumatologists and surgeons in the post-operative management of aortitis.

POS0804 OUTCOMES OF SURGICALLY RESECTED THORACIC AORTITIS: RESULTS FROM A PROSPECTIVE MULTI-CENTRE REGISTRY

Background:Inflammatory thoracic aortitis is a cause of aneurysm and is often diagnosed incidentally following aortic replacement surgery. It may be due to systemic diseases such as giant cell arteritis (GCA) and Takayasu arteritis (TAK) or a topographically isolated variant termed clinically isolated aortitis (CIA). While CIA is identified in 2-12% of thoracic aortic surgical pathology,1 its short-term post-operative outcome is unknown.Objectives:This study was undertaken to examine the short-term post-operative mortality and morbidity of patients with surgically resected thoracic aortitis compared to those with non-inflammatory thoracic aortic aneurysms and assess if outcomes varied based on post-operative corticosteroid therapy.Methods:The study was based at three tertiary referral hospitals in Sydney, Australia, for the years 2004-2018. Prospectively collected data was accessed from a national surgical registry for all patients who underwent thoracic aortic aneurysm replacement. Aortitis patients were identified from histopathology records.Inpatient medical records were reviewed to categorise aortitis cases as CIA, GCA, TAK or other aortitis and to audit the use of corticosteroids. Outcomes were compared between aortitis cases and the non-inflammatory cohort using logistic regressions, controlling for age, sex, year of procedure, and hospital.Results:41 aortitis cases were identified from the cohort of 1119 surgical patients (3.7%). 27 (66%) met criteria for CIA, 11 (27%) for GCA, 2 (5%) for TAK, and 1 (2%) for Other. 8 (20%) received corticosteroid therapy for aortitis.Compared with non-inflammatory patients, the aortitis cohort was predominantly female (54% vs 28%, p &lt; 0.01), older (mean 70 vs 62 years, p &lt; 0.01), and had higher rates of hypertension (83% vs 67%, p = 0.03) and pre-operative immunosuppression (10% vs 1%, p &lt; 0.01).There was no difference (p &gt; 0.05) between the aortitis and the non-aortitis groups for 30-day mortality (7% vs 7%), composite significant morbidity (15% vs 22%), composite infection (10% vs 6%), return to theatre for bleeding (7% vs 13%), stroke (5% vs 5%) or readmission (12% vs 10%). Similar results were also seen for CIA and the aortitis subgroup who did not receive corticoid steroid therapy when compared to the non-aortitis group (table 1).Table 1.30-day mortality and morbidityNon-aortitisAll aortitisAortitis, not treated for aortitisCIAn=1078n=41n=33n=27%n%n%n%nMortality770736241Stroke557526241Return to theatre bleed13137736241Composite morbidity22241156155113Composite infection66910493113Readmission10108125124113Note: Differences in outcomes between the aortitis groups and the no aortitis group were modelled using logistic regression, controlling for age, sex, year of procedure, and hospital. There were no statistically significant differences in outcomes (p &gt; 0.05).CIA; clinically isolated aortitis. Composite morbidity; an outcome comprised of return to theatre for bleeding, stroke, new renal failure, acute myocardial infarction or aortic dissection. Composite infection; an outcome comprised of 30-day pneumonia, septicaemia, deep sternal wound infection or deep thoracotomy infection.Conclusion:The finding of thoracic aortitis following aortic replacement surgery does not affect short-term post-operative mortality or morbidity. Corticosteroid therapy in the immediate post-operative period did not impact upon short-term outcomes.

Comparison of the damage to aorta wall in aortitis versus noninflammatory degenerative aortic diseases

BackgroundNot rarely aortitis is firstly identified in thoracic aorta aneurysm/dissection specimens only by histopathology in the absence of clinical evidence of systemic inflammatory disease emphasizing the importance of histology for the diagnosis of aortitis. Regardless of the improvement of the pathological assessment of aortic diseases by the recent consensus statements on surgical pathology of the aorta, histology can be confusing since medial degenerative changes (MDC) can be prominent in a background where inflammation is sometimes limited. This raises the question of the role of aging or other degenerative process versus the role of inflammation in the damage to aorta wall. Patients and MethodsIn this study, besides inflammation, we evaluated aorta samples from aortitis cases focusing on the histological scoring of MDC. In this retrospective single center study, we retrieved 719 cases of ascending aorta aneurysms or dissections operated on from January 2010 until June 2018. MDC (elastic fiber fragmentation and/or loss, smooth muscle nuclei loss, mucoid extracellular matrix accumulation intralemellar or translamellar) were estimated using a scoring system derived from that of the consensus statement. Noninfectious aortitis group versus age-matched non-inflammatory degenerative aortic disease group were compared. ResultsNoninfectious aortitis was pathologically diagnosed in 62 patients (8.6%). Among the 62 noninfectious aortitis patients, 47 patients (75.8%) had aortitis identified pathologically prior to the clinical diagnosis. Higher MDC scores were observed at all aortic sizes in aortitis group versus non-aortitis group, especially for elastic fiber damage and smooth muscle cell loss. ConclusionsAortitis is remarkably associated with severe damage to the aorta wall resulting in advanced MDC scores. Inflammatory process is responsible for higher MDC in the aorta wall than aging or other degenerative process.

Aortitis - An Interdisciplinary Challenge.

The term 'aortitis' comprises a heterogeneous spectrum of diseases, with varied etiology and clinical presentations, whose common characteristic is the inflammation of the aortic wall. Since aortitis can mimic almost all common cardiovascular disorders, its clinical recognition remains a challenge. Some cases of aortitis remain undetected for a long time and may be diagnosed after severe life-threatening complications have already arisen. The diagnosis of aortitis is based on the presence of homogeneous circumferential thickening of the aortic wall detected on aortic imaging, or typical histological features in combination with clinical findings and laboratory parameters. Management of aortitis is usually conservative (immunosuppressive drugs in noninfectious aortitis; antimicrobial drugs in infectious). However, if vascular complications such as aortic aneurysm, rupture, or steno-occlusive events appear, aortic surgery or endovascular therapy may be required. This review article summarizes the current knowledge regarding the etiology, clinical presentation, diagnosis, and treatment of inflammatory diseases of the aorta to promote better clinical management of these entities.

P176 A retrospective analysis of aortitis cases from radiology reports in a London teaching hospital: implications for screening and management

Abstract Background Aortitis is a heterogeneous rare condition causing aortic inflammation, often due to infectious or autoimmune aetiology. Its presentation, diagnosis and optimal management remain poorly understood, often requiring multidisciplinary input. With the advent of improved imaging techniques, an increasing number of radiology reports are identifying features of vasculitis necessitating clinical investigation. We investigated how cases identified on imaging with features of aortitis reflected clinical diagnosis and treatment. We further examined whether radiological detection of aortitis enables more rapid management decisions to improve outcomes. Methods A search was conducted of all radiology reports from St George’s Hospital between 2008 and 2018 for key words indicating “aortitis”, including ‘hyperintense vessel uptake’ and ‘periaortic inflammation.’ From 15,963 initial results, 80 reports were identified with radiological suspicion of aortitis. Results Of 80 cases, 36/80 were diagnosed with autoimmune or idiopathic inflammatory pathology: 9 retroperitoneal fibrosis, 8 idiopathic, 7 Takayasu’s, 4 giant cell arteritis, 2 inflammatory aneurysms, 2 Behçet's, 2 IgG4 disease, 1 lupus, 1 granulomatosis with polyangiitis. Diagnosis of autoimmune or idiopathic aortitis was based on symptomatology, imaging, serology, vessel biopsy, and treatment response. 14/80 had infective aortitis: 11 had positive microbiology and 3 demonstrated empirical antibiotic response. Table 1 shows demographics. 12/80 had atheromatous disease. 18/80 were not further investigated due to comorbidity, lack of correlation with symptoms, or absence of follow-up. Initial imaging modalities with the highest yield were computed tomography (CTs) with aorta protocols and CTs of thorax/abdomen/pelvis. The most useful radiology report terms included: aortitis, periaortic inflammation, Takayasu’s, and hyperintense vessel uptake. Conclusion Our study is the first retrospective case analysis, to our knowledge, of more than 15,000 radiology reports used as a starting point to evaluate for aortitis, identifying a large dataset with a broad case-mix. It contrasts with current literature identifying cases histologically and post-operatively. We found that radiology can be a useful early alert for possible diagnoses which require further assessment. Consequently, we developed an alerting system within our radiology department based on the search terms and imaging modalities identified. This links to a multidisciplinary meeting including vascular and rheumatology, so highlighted cases are discussed early. Disclosures K. Townsend: None. G. Cattini: None. K. Moss: None. K. Stenson: None. P. Holt: None. R. Morgan: None. N. Sofat: None.

Mechanism and biomarkers in aortitis--a review.

Aortitis can be the manifestation of an underlying infectious or noninfectious disease process. An autoimmune cause is suggested in a large proportion of noninfectious causes. Similar to other autoimmune diseases, the pathophysiology of aortitis has been investigated in detail, but the etiology remains unknown. Most cases of aortitis often go undetected for a long time and are often identified at late stages of the disease. Recent advances in imaging techniques have significantly improved the diagnosis of aortitis. However, significant challenges associated with the imaging techniques limit their use. Several routine inflammation-based markers, such as erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), and inflammatory cytokines, are nonspecific and, therefore, have limited use in the diagnosis of aortitis. The search for more specific serum biomarkers, which can facilitate detection and progression is under progress. Several autoantibodies have been identified, but assigning their role in the pathogenesis as well as their specificity remains a challenge. The current review addresses some of these issues in detail. KEY MESSAGES: • Noninfectious aortitis is an autoimmune disease. • Several biomarkers, including cytokines and autoantibodies, are increased in aortitis. • Imaging techniques, commonly used to detect aortitis, are associated with the high cost and technical challenges. • There is a need to develop low-cost biomarker-based detection tools. • The knowledge of biomarkers in aortitis detection is discussed.

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

Objective:We sought to characterize microbiomes of thoracic aortas from patients with non-infectious aortitis due to giant cell arteritis (GCA) and clinically isolated aortitis (CIA) and to compare them to non-inflammatory aorta aneurysm controls. We also compared microbiomes from concurrently processed and separately reported temporal arteries (TA) and aortas.Methods:From 220 prospectively enrolled patients undergoing surgery for thoracic aorta aneurysm, 49 were selected. Inflammatory and non-inflammatory cases were selected based on ability to match for age (+/-10 years), gender, and race. Biopsies were collected under aseptic conditions and snap-frozen. Taxonomic classification of bacterial sequences was performed to the genus level and relative abundances were calculated. Microbiome differential abundances were analyzed by principal coordinates analysis.Results:Forty-nine patients with thoracic aortic aneurysms (12 CIA, 14 GCA, 23 non-inflammatory aneurysms) were enrolled. Alpha (P=0.018) and beta (P=0.024) diversity differed between specimens from aortitis cases and controls. There were no significant differences between CIA and GCA (P>0.7). The largest differential abundances between non-infectious aortitis and non-inflammatory control samples included Enterobacteriaceae, Phascolarctobacterium, Acinetobactor, Klebsiella, and Prevotella. Functional metagenomic predictions with PICRUSt revealed enrichment of oxidative phosphorylation and porphyrin metabolism pathways and downregulation of transcription factor pathways in aortitis compared to controls. Microbiomes of aortic samples differed significantly from temporal artery samples from a companion study, in both control and GCA groups (P=0.0002).Conclusion:Thoracic aorta aneurysms, far from being sterile, contain unique microbiomes that differ from those found in temporal arteries. The aorta microbiomes are most similar between aneurysms that were associated with inflammation, GCA, and CIA, but differed from those associated with non-inflammatory etiologies. These findings are promising in that they indicate that microbes may play a role in the pathogenesis of aortitis-associated aneurysms or non-inflammatory aneurysms by promoting or protecting against inflammation. However, we cannot rule out that these changes are related to alterations in tissue substrate that favor secondary changes in microbial communities.

Infectious Aortitis: Could Early Radiology Improve Patient Outcome?

Aortitis is a rare condition and easily overlooked. It is defined as infectious or non-infectious inflammation of the aortic wall. This report describes two cases of aortitis, demonstrating the diagnostic difficulty and how diagnostic delay could have been reduced if early radiology had been performed. Due to the nature of aortitis, patient outcome can be improved considerably by timely diagnosis and treatment.LEARNING POINTSAortitis is rarely suspected due to its vague symptoms, but diagnosis is aided by computed tomography (CT) of the thorax and abdomen.A delay in aortitis diagnosis can be life threatening; however, early radiology can ensure timely diagnosis and considerably improve patient outcome.Aortitis should always be suspected in cases with immunosuppression and systemic Salmonella infection, and a CT of the thorax and abdomen should be performed.

Young Investigator: A Rare Case of Crohnʼs Disease-Associated Aortitis

RESULTS: A 33 year-old woman with history of Crohn's disease complicated by enteropathic arthropathies presented with symptoms of complete gastrointestinal obstruction. She had history of ongoing ileocolitis despite therapy with certolizumab. Due to persistent obstruction symptoms, she underwent an uneventful laparoscopic ileocecectomy. On post-operative day number two, she developed fever, chest pain, tachycardia and shortness of breath. Her WBC was elevated to 10 K/microL, with CRP 10 mg/L, total IgG and IgG2 noted to be elevated with normal IgG4 levels. Interferon release assay for tuberculosis was negative. A chest CT revealed ascending aortitis, which was later confirmed with MR angiography. She was started on high-dose corticosteroids and ultimately transitioned to infliximab (5 mg/kg) and subcutaneous methotrexate with resolution of aortitis on follow-up imaging. While her aortitis has remained in remission, her intestinal Crohn's symptoms and arthralgias are recurring despite recently increasing infliximab dose to 10mg/kg every 6 weeks, with therapeutic levels and no evidence of anti-infliximab antibodies. CONCLUSION(S): Extraintestinal manifestations (EIM) of inflammatory bowel disease are commonly found in approximately one-third of patients. They typically manifest as peripheral or axial arthropathies, dermatologic manifestations including pyoderma gangrenosum and erythema nodosum, ocular involvement including uveitis or episcleritis, and primary sclerosing cholangiopathy. While very uncommon, there also appears to be some degree of overlap between Crohn's disease and large vessel vasculitides. Initial evaluation should exclude diagnoses of Takayasu's arteritis, Behcet's disease, sarcoidosis, ankylosing spondylitis, and connective tissue disorders. Infectious etiologies such as tuberculosis and syphilis should also be eliminated. Upon exclusion of these disease processes causing aortic inflammation, the diagnosis of a Crohn's-related EIM should be considered. Only a few cases of aortitis associated with Crohn's disease have been reported in the literature but this appears to be a very rare phenomenon. Severe cases of aortitis carry the risk of aortic rupture when inflammation is left untreated. Fortunately, aortitis appears to be readily responsive to high dose corticosteroid therapy, as was the case in our patient. If significant valvular disease is present (i.e. severe aortic regurgitation), this may require valvular replacement surgery. Interestingly, there have been reports of Crohn's-related aortitis developing in the setting of anti-TNF therapy, again requiring corticosteroid therapy for resolution. As such, we will need to monitor our patient closely to detect any signs of recurrent aortitis.

Tuberculous aortitis, an unusual presentation of tuberculosis

51-year-old female who recently emigrated from Mexico with past history of coronary artery disease (CAD) status post coronary artery bypass grafting (CABG) two years prior who presented with chest pain. She reported diffuse anterior chest pain that radiated to the back. She also reported a 4 kg weight loss, night sweats, chills and fevers over the same time period. In the emergency department her EKG was normal, cardiac enzymes were negative and preliminary CTA report was concerning for possible aortitis vs aortic hematoma. Her ESR and CRP were elevated but autoimmune work-up including ANCA and ANA was negative, raising suspicion for an underlying infectious etiology. The patient had a positive PPD skin test with 17 mm induration, a positive quantiferon test and chest X-ray showed numerous lung nodules bilaterally, consistent with a diagnosis of tuberculosis. Transesophageal echocardiogram showed regional thickening of ascending aorta with the major differential being aortitis vs intramural hematoma vs chronic aortic dissection. Her records from her CABG done in Mexico were obtained and described a porcelain aorta, which further supported a diagnosis of TB aortitis. Cardiothoracic surgery was consulted in light of these results, and they believed that the patient’s aortic thickening was caused by diffuse aortitis. Thus she was diagnosed with aortitis secondary to her underlying tuberculosis. Most cases of aortitis are noninfectious in nature. However, although rare, infectious causes should always be considered due to associated high morbidity and mortality as well as potential reversibility. Tuberculosis as a cause of aortitis is very rare even in developing countries. Tuberculous aortitis should always be considered among patients with aortitis or aortic hematomas, especially if they are among high risk population for tuberculosis or have a history of untreated tuberculosis.

A case series of surgically diagnosed idiopathic aortitis in a Canadian centre: a retrospective study.

Idiopathic aortitis became recognized relatively recently, and the body of knowledge concerning this condition is scarce. We aimed to determine the frequency of idiopathic aortitis in aortic specimens, the clinical, laboratory and radiologic characteristics at diagnosis and during follow-up, and the approach to investigation, treatment and monitoring taken by the treating physicians. We identified cases of aortitis diagnosed on pathological specimens of the aorta between Jan. 1, 2003, and July 31, 2013, at The Ottawa Hospital by reviewing the hospital's pathology database. Charts of identified patients were reviewed, and data on patient demographic characteristics, clinical features, laboratory and imaging tests, treatment and outcomes were analyzed. A total of 684 aortic specimens were analyzed during the study period; 47 cases of aortitis were identified, 32 of which were idiopathic. Twenty-one patients (66%) had complete imaging of branch vessels at baseline, 16 (76%) of whom had additional aortic or branch vessel lesions. Twelve patients (38%) received corticosteroids postoperatively. Over a mean follow-up period of 47.5 months, among the 12 patients (38%) who had complete imaging of branch vessels at least once, new aortic or branch lesions were diagnosed in 5 (42%); 3/32 patients (9%) required additional vascular surgery; and a new systemic condition was diagnosed in 2/32 (6%). Idiopathic aortitis is commonly discovered incidentally on examination of the pathological specimen following ascending aortic aneurysm repair. No guidelines exist for the investigation, treatment and follow-up of this condition, resulting in great variability of practice. Good-quality prospective studies are needed to address the many unanswered clinical questions regarding idiopathic aortitis and to allow formulation of more definitive recommendations.

Role of 18F-FDG PET/CT in the diagnosis of inflammatory and infectious vascular disease.

This review article discusses the utility of 18F-FDG PET/CT in diagnosis and management of vascular disease. We stress usefulness of this method in large vessel inflammation and infection. In our work we based on the literature analysis and clinical cases diagnosed in our institution by use of 18F-FDG PET/CT. The literature exploration was focusing on vascular inflammation and infections and 18-FDG PET. The search was performed on PubMed database and cross referencing. We present the practical review with several images of vascular diseases like: Takayasu arteritis, giant cell arteritis, vascular graft infections, abdominal aortic aneurysm infections and cases of aortitis and periaortitis. From this work inflammation associated with atheromatic process and vulnerable atherosclerotic plaque we excluded. 18F-FGD PET/CT is a sensitive metabolic, reliable, non-invasive imaging modality suitable for diagnosis and follow-up of inflammation and infections in vascular system.

An elevated IgG4 response in chronic infectious aortitis is associated with aortic atherosclerosis

Recently, it was shown that infectious bacterial aortitis can stimulate an elevated IgG4+ plasma cell response in the vessel wall, which could mimic IgG4 aortitis/periaortitis. However, the factors that are associated with an elevated IgG4+ plasma cell response in infectious aortitis are unclear. To ascertain these factors, 17 cases of infectious aortitis and 6 cases of non-infectious severe abdominal aortic atherosclerosis were assessed for the magnitude of IgG4+ plasma cell response. The degree of IgG4+ plasma cell infiltration was determined by immunohistochemistry. Infectious cases were subcharacterized as chronic (>3 weeks duration) or acute (<3 weeks duration) based on the duration of symptoms, and as involving either the ascending aorta or the distal aorta, ie, the descending thoracic and/or abdominal aorta. There was a 5–16-fold greater degree of IgG4+ plasma cell infiltration in the chronic distal infectious aortitis group compared with the other three infectious aortitis groups (P≤0.0007), and compared with non-infectious severe abdominal aortic atherosclerosis (P<0.0008). This resulted in a greater IgG4/IgG ratio in the chronic distal infectious aortitis group compared with the acute ascending and acute distal infectious aortitis groups (P<0.03). The degree of IgG4+ plasma cell infiltration in chronic distal infectious aortitis overlaps with that seen in the aortitis and periaortitis of IgG4-related disease. In the chronic infectious aortitis cases, the degree of IgG4+ plasma cell infiltration was more intense in patients with moderate to severe aortic atherosclerosis compared with those patients with less aortic atherosclerosis (P=0.007). These findings indicate that an elevated IgG4+ plasma cell response occurs in the descending thoracic and abdominal aorta in the setting of chronic bacterial infectious aortitis and pre-existing atherosclerosis. This inflammatory response to chronic infection in atherosclerosis-laden aortas may have implications for the development of IgG4-rich inflammatory atherosclerotic aortic aneurysms.

Aortitis in patients with psoriatic arthropathy: report of two cases and review of the literature

Aortitis, which is well described in patients with other spondyloarthritides, has been rarely cited in relation to psoriatic arthropathy (PsA). Two patients with known PsA, who developed aortitis, are reported herein. The PubMed database was searched using the following keywords: aortitis, Takaysu arteritis, PsA, ankylosing spondylitis, reactive arthritis, inflammatory bowel disease. The relevant articles were critically reviewed and pertinent data organized. Analysis of 5 cases of aortitis in patients with PsA, including the 2 cases reported herein, revealed no specific pattern of PsA joint involvement in the patients who developed aortitis. All aortic segments can be involved and complications, such as insufficiency of the aortic valve and stenosis of the major aortic branches, have been described. The genetic association involving the IL12B locus may be involved in the clinical association of aortitis and spondyloarthritis.

Ascending aortitis: a clinicopathological study of 21 cases in a series of 300 aortic repairs

There are few single-institution clinicopathological series of aortitis. In this study, all ascending aneurysms were prospectively evaluated pathologically with ≥6 aortic sections over a 6-year period.Of 300 ascending aortic resections, there were 21 cases of aortitis (7%), in 11 women and 10 men (mean 67, range 41-88 years). There were 19 patients with aneurysms, and two patients with sclerosing periaortitis, clinically suspected to have intramural haematoma. Of the 19 patients with aneurysms (11 women), two had prior temporal arteritis, one ankylosing spondylitis, one IgA nephropathy, one undifferentiated autoimmune disease, one Lyme disease, and one fibromyalgia. In only two patients was aortitis suspected before surgery as the cause of aneurysm. Four patients developed distal aortic aneurysm requiring repeat surgery. Valve replacement or repair was necessary in nine patients, and two patients died after surgery. There were no significant differences between patients with and without autoimmune disease. The histological features were necrotising aortitis in 18 of 19 patients with aneurysmal aortitis, and there was one case of non-necrotising aortitis. One valve showed autoimmune valvulitis, congenitally bicuspid associated with ankylosing spondylitis. Necrotising aortitis was classified as acute (n = 5), healing (n = 9), and healed (n = 4). Acute necrotising aortitis was associated with need for valve replacement (p = 0.01) and younger age (p = 0.01). The healed phase had subtle histological features, sparse medial inflammation, marked medial attenuation, and chronic adventitial inflammation. Two patients with periaortitis demonstrated marked fibroinflammatory thickening of the adventitia with histological features typical of IgG4-related disease; neither had systemic symptoms. Ascending aortitis is histologically diverse, most frequently of the medial necrotising type, and is usually not suspected pre-operatively. Awareness of the histological spectrum is necessary for pathological diagnosis.

Frequency of immunoglobulin G4‐related aortitis in cases with aortic resection and their clinical characteristics compared to other aortitises

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.

Surgical Management of Aortitis with Early Aneurysmal Dilation

Aortitis is a rare and serious condition that requires expedient surgical evaluation. Diagnosis is generally made by computed tomography (CT). Surgery is most often performed when significant aneurysmal changes have already occurred. Outcomes of early surgical management of aortitis with early aneurysmal dilation have not been reported previously. A retrospective review of open abdominal aortic repairs performed from 1999 to 2009 at a single center was done from a prospectively collected database. Patients with a confirmed radiographic appearance of aortitis and treated surgically were selected. Demographic, clinical, and surgical data of patients with aortitis showing early aneurysmal changes (aortic diameter <4cm) were then analyzed. All aortitis cases with >4-cm aortic diameters and with prosthetic aortic grafts were excluded. During the observation period, 421 open abdominal aortic repairs were performed. Of these, 10 (2.4%) were identified as having primary aortitis without significant aneurysmal changes. The mean age of the patients was 62 (range 48-77) years. There were 6 (60%) men and 4 (40%) women in the cohort. Four patients (40%) had culture-negative aortitis, whereas 6 (60%) had positive microbial cultures at the time of diagnosis. Paravisceral involvement was seen in 8 (80%) cases. All patients underwent in situ repair with aortic homografts. Mean operative time was 348 minutes and mean estimated blood loss was 2475 mL. Median follow-up time was 23.1 months with a range of 1.7-51.4 months. Operative mortality was 0%, and 1 late death occurred at 23 months postoperatively. There were 9 significant in-hospital (30-day) events occurring in 5 patients, including 3 cardiovascular events, 2 pulmonary events, 3 acute renal failures, and 1 deep surgical site infection. Aortitis is an uncommon indication for aortic repair. Infectious aortitis is most commonly confirmed by microbiologic studies, but a significant number of cases have no demonstrable microbial source. Outcomes after early surgical management for aortitis with small aneurysms demonstrated improved mortality when compared with series reviewing outcomes in aortitis patients with large mycotic aneurysms.

CT angiographic imaging characteristics of thoracic idiopathic aortitis

BackgroundIdiopathic aortitis (IA) is characterized by giant cell or lymphoplasmacytic inflammation of aorta without a secondary cause. ObjectiveWe undertook a retrospective case–control study to identify characteristic CT angiographic findings in these patients and to correlate them with known atherosclerotic risk factors. MethodsIA cases and controls with noninflammatory aneurysm (control group I) and patients with secondary aortitis (control group II) were identified with a pathology database. Preoperative CT angiographic images of thoracic aorta were reviewed. Diameter of thoracic aorta, wall thickness, and calcification were measured at various sites. Traditional atherosclerotic risk factors were identified from case records and included hypertension, hyperlipidemia, diabetes mellitus, and smoking. ResultsTwenty-two idiopathic aortitis cases were compared with 18 patients in control group I and 16 patients in control group II. No differences were found in prevalence of hypertension and diabetes, but hyperlipidemia was more prevalent in the control group I than in cases (72% vs 36%; P = .03). Current smoking was more prevalent in cases (24%) than for patients in control group I (6%) and group II (19%) but not statistically significant (P = .18 and .69, respectively). Thoracic aortic diameters at various points were significantly larger in cases than for patients in control group I. Calcification was more frequent in cases than for patients in control group II. No differences in wall thickness were found. No meaningful correlation was observed between atherosclerotic risk factors and aortic diameter and calcification scores. ConclusionsPatients with IA have significantly larger and more diffuse dilatation of the thoracic aorta than patients with noninflammatory aneurysms.

Aortite thoraco-abdominale au cours d’une spondylarthrite ankylosante : un nouveau cas et revue de la littérature

We report a case of aortitis in a patient with ankylosing spondylitis revealed by an unexplained persistent inflammation. The diagnosis of ankylosing spondylitis was retained in a 64-year-old woman suffering from inflammatory back and neck pain combined with buttock pain relieved by anti-inflammatory drugs (NSAIDs) since 2004 and more recent bilateral heel pain in the morning since 2006; sacroiliitis was grade 3 on the right and grade 2 on the left (modified New-York criteria). The patient had remained asymptomatic from April 2006 to 2007 with NSAID as needed. Nevertheless, biological inflammation persisted: erythrocyte sedimentation rate 44 to 55 mm/h, activated protein C 34 to 90 mg/L. Complementary examinations are negative: bilateral temporal artery biopsy, endoscopy with duodenal biopsy looking for Tropheryma whipplei. The thoraco-abdominal and pelvic CT scan revealed aortitis extending from the abdominal aorta to the iliac axis. Treatment with prednisone 0.5 mg/kg was started to decrease the inflammatory aortitis. The most "classical" cardiovascular damage observed in spondylitis is aortic insufficiency and conduction disturbances. The first cases of aortitis were reported in 1958. Inflammatory vascular disease should be evoked as a possible diagnosis in patients with ankylosing spondylitis the presenting an unexplained biological inflammation (ESR and CRP).