Clinical and pathological spectrum of aortitis in a Chinese cohort.

BACKGROUND: This study aimed to explore the clinical and pathological features of aortitis in China, which is a rare disease that is often overlooked preoperatively. METHODS: We 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. RESULTS: Out 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. CONCLUSIONS: Half 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.

Long-Term Outcome and Prognosis of Noninfectious Thoracic Aortitis.

BACKGROUND: Aortitis is a group of disorders characterized by the inflammation of the aorta. The large-vessel vasculitides are the most common causes of aortitis. Aortitis long-term outcomes are not well known. OBJECTIVES: The purpose of this study was to assess the long-term outcome and prognosis of noninfectious surgical thoracic aortitis. METHODS: This was a retrospective multicenter study of 5,666 patients with thoracic aorta surgery including 217 (3.8%) with noninfectious thoracic aortitis (118 clinically isolated aortitis, 57 giant cells arteritis, 21 Takayasu arteritis, and 21 with various systemic autoimmune disorders). Factors associated with vascular complications and a second vascular procedure were assessed by multivariable analysis. RESULTS: Indications for aortic surgery were asymptomatic aneurysm with a critical size (n = 152 [70%]), aortic dissection (n = 28 [13%]), and symptomatic aortic aneurysm (n = 30 [14%]). The 10-year cumulative incidence of vascular complication and second vascular procedure was 82.1% (95% CI: 67.6%-90.6%), and 42.6% (95% CI: 28.4%-56.1%), respectively. Aortic arch aortitis (HR: 2.08; 95% CI: 1.26-3.44; P = 0.005) was independently associated with vascular complications. Descending thoracic aortitis (HR: 2.35; 95% CI: 1.11-4.96; P = 0.031) and aortic dissection (HR: 3.08; 95% CI: 1.61-5.90; P = 0.002) were independently associated with a second vascular procedure, while treatment with statins after aortitis diagnosis (HR: 0.47; 95% CI: 0.24-0.90; P = 0.028) decreased it. After a median follow-up of 3.9 years, 19 (16.1%) clinically isolated aortitis patients developed features of a systemic inflammatory disease and 35 (16%) patients had died. CONCLUSIONS: This multicenter study shows that 82% of noninfectious surgical thoracic aortitis patients will experience a vascular complication within 10 years. We pointed out specific characteristics that identified those at highest risk for subsequent vascular complications and second vascular procedures.

Single centre experience of 120 patients with non-infectious aortitis: Clinical features, treatment and complications.

BACKGROUND: Aortitis is an important form of vasculitis with significant risk of complications. Very few studies have provided detailed clinical phenotyping across the whole disease spectrum. Our primary aim was to look the clinical features, management strategies and complications associated with non-infectious aortitis. METHODS: A retrospective review was performed on patients with diagnosis of noninfectious aortitis at the Oxford University hospitals NHS Foundation Trust. Clinicopathologic features were recorded including demographics, presentation, aetiology, laboratory, imaging findings, histopathology, complications, treatment, and outcome. RESULTS: We report the data on 120 patients (59% females). Systemic inflammatory response syndrome constituted the most common presentation (47.5%). 10.8% were diagnosed following a vascular complication (dissection or aneurysm). All patients (n = 120) had raised inflammatory markers (median ESR 70.0 mm/h and CRP 68.0 mg/L). Isolated aortitis subgroup (15%) had significantly higher likelihood of presenting with vascular complications and challenging to diagnose due to non-specific symptoms. Prednisolone (91.5%) and methotrexate (89.8%) were the most used treatment. 48.3% developed vascular complications during the disease course including ischaemic complications (25%), aortic dilatation and aneurysms (29.2%) and dissection (4.2%). Risk of dissection was higher in the isolated aortitis subgroup at 16.6% compared to all other types of aortitis at 1.96%. CONCLUSION: Risk of vascular complications is high in non-infectious aortitis patients during disease course, hence early diagnosis and appropriate management is key. DMARDs such as Methotrexate appear to be effective, nonetheless there remain gaps in evidence for longer-term management of relapsing disease. Dissection risk seems much higher for patients with isolated aortitis.

The epidemiology of pathologically confirmed clinically isolated aortitis: a North American population-based study.

OBJECTIVES: Clinically isolated aortitis (CIA) refers to inflammation of the aorta without signs of systemic vasculitis or infection. Population-based data on the epidemiology of CIA in North America is lacking. We aimed to investigate the epidemiology of pathologically confirmed CIA. METHODS: Residents of Olmsted County, Minnesota were screened for thoracic aortic aneurysm procedures with current procedural terminology codes between January 1, 2000, and December 31, 2021, using the resources of the Rochester Epidemiology Project. The medical records of all patients were manually reviewed. CIA was defined as histopathologically confirmed active aortitis diagnosed by evaluation of aortic tissue obtained during thoracic aortic aneurysm surgery in the absence of any infection, rheumatic disease, or systemic vasculitis. Incidence rates were age and sex adjusted to the 2020 United States total population. RESULTS: Eight incident cases of CIA were diagnosed during the study period; 6 (75%) of them were female. Median (IQR) age at diagnosis of CIA was 78.3 (70.2-78.9) years; all were diagnosed following ascending aortic aneurysm repair. The overall age and sex adjusted annual incidence rate of CIA was 8.9 (95% CI, 2.7-15.1) per 1,000,000 individuals over age 50 years. The median (IQR) duration of follow-up was 8.7 (1.2-12.0) years. The overall mortality compared to the age and sex matched general population did not differ (standardised mortality ratio: 1.58; 95% CI, 0.51-3.68). CONCLUSIONS: This is the first population-based epidemiologic study of pathologically confirmed CIA in North America. CIA predominantly affects women in their eighth decade and is quite rare.

Retrospective Multicenter Study Comparing Infectious and Noninfectious Aortitis.

BACKGROUND: Determining the etiology of aortitis is often challenging, in particular to distinguish infectious aortitis (IA) and noninfectious aortitis (NIA). This study aims to describe and compare the clinical, biological, and radiological characteristics of IA and NIA and their outcomes. METHODS: A multicenter retrospective study was performed in 10 French centers, including patients with aortitis between 1 January 2014 and 31 December 2019. RESULTS: One hundred eighty-three patients were included. Of these, 66 had IA (36.1%); the causative organism was Enterobacterales and streptococci in 18.2% each, Staphylococcus aureus in 13.6%, and Coxiella burnetii in 10.6%. NIA was diagnosed in 117 patients (63.9%), mainly due to vasculitides (49.6%), followed by idiopathic aortitis (39.3%). IA was more frequently associated with aortic aneurysms compared with NIA (78.8% vs 17.6%, P < .001), especially located in the abdominal aorta (69.7% vs 23.1%, P < .001). Crude and adjusted survival were significantly lower in IA compared to NIA (P < .001 and P = .006, respectively). In the IA cohort, high American Society of Anesthesiologists score (hazard ratio [HR], 2.47 [95% confidence interval {CI}, 1.08-5.66]; P = .033) and free aneurysm rupture (HR, 9.54 [95% CI, 1.04-87.11]; P = .046) were significantly associated with mortality after adjusting for age, sex, and Charlson comorbidity score. Effective empiric antimicrobial therapy, initiated before any microbial documentation, was associated with a decreased mortality (HR, 0.23, 95% CI, .08-.71]; P = .01). CONCLUSIONS: IA was complicated by significantly higher mortality rates compared with NIA. An appropriate initial antibiotic therapy appeared as a protective factor in IA.

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. 62 years, 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.

Spectrum and Outcome of Noninfectious Aortitis.

OBJECTIVE: To assess the spectrum and long-term outcome of patients with noninfectious aortitis. METHODS: We performed a retrospective multicenter study of 353 patients (median age at diagnosis was 62 [IQR 46-71] yrs and 242 [68.6%] patients were women) with noninfectious aortitis. Factors associated with vascular complications were assessed in multivariate analysis. RESULTS: We included 136 patients with giant cell arteritis (GCA), 96 with Takayasu arteritis (TA), 73 with clinically isolated aortitis (CIA), and 48 with aortitis secondary to inflammatory diseases (including Behçet disease, relapsing polychondritis, IgG4-related disease, Cogan syndrome, ankylosing spondylitis). After a median follow-up of 52 months, vascular complications were observed in 32.3%, revascularizations in 30% of patients, and death in 7.6%. The 5-year cumulative incidence of vascular complications was 58% (95% CI 41-71), 20% (95% CI 13-29), and 19% (95% CI 11-28) in CIA, GCA, and TA, respectively. In multivariate analysis, male sex (HR 2.10, 95% CI 1.45-3.05, P < 0.0001) and CIA (HR 1.76, 95% CI 1.11-2.81, P = 0.02) were independently associated with vascular complications. CONCLUSION: Noninfectious aortitis accounts for significant morbidity and mortality. CIA seems to carry the highest rate of vascular complications.

Symptomatic aortitis at giant cell arteritis diagnosis: a prognostic factor of aortic event.

BACKGROUND: Giant cell arteritis (GCA) is frequently associated with aortic involvement that is likely to cause life-threatening structural complications (aneurysm, dissection). Few studies have investigated the occurrence of these complications, and no predictive factor has been identified so far. The aim of this study was to investigate factors associated with the risk of aortic complications in a cohort of GCA aortitis. METHODS: Data of all patients managed with aortitis (CT or 18 FDG PET) at the diagnosis of GCA in five hospitals from May 1998 and April 2019 were retrospectively collected. Clinical features were compared according to the presence of aortitis symptoms. The predictive factors of occurrence or aggravation of aortic structural abnormalities were investigated. RESULTS: One hundred and seventy-one patients with GCA aortitis were included; 55 patients (32%) had symptoms of aortitis (dorsal/lumbar/abdominal pain, aortic insufficiency) at diagnosis. The median follow-up was 38 months. Aortic complications occurred after a median time of 32 months. There were 19 new aortic aneurysms or complications of aneurysm and 5 dissections. Survival without aortic complication was significantly different between the symptomatic and non-symptomatic groups (Log rank, p = 0.0003). In multivariate analysis the presence of aortitis symptoms at diagnosis (HR 6.64 [1.95, 22.6] p = 0.002) and GCA relapse (HR 3.62 [1.2, 10.9] p = 0.02) were factors associated with the occurrence of aortic complications. CONCLUSION: In this study, the presence of aortitis symptoms at the diagnosis of GCA aortitis and GCA relapse were independent predictive factors of occurrence of aortic complications during follow-up.

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.

Aortitis: an update.

PURPOSE OF REVIEW: Aortitis is the inflammation of the aorta due to various causes. Clinical presentations vary as well as the imaging findings. Exact pathogenetic mechanisms or triggering factors, as well as the best diagnostic and monitoring modalities and treatment strategies, are yet to be elucidated. We reviewed recent studies in aortitis and associated diseases. RECENT FINDINGS: Multiple cohort studies reporting long-term outcomes in patients with noninfectious aortitis were recently published. Comparative features of isolated aortitis were described. Six angiographic clusters for giant cell arteritis and Takayasu have been identified. New classification criteria have been proposed for IgG4-related disease by a data-driven method. The ultrasonographic slope sign and a halo score were described as specific imaging parameters in giant cell arteritis. The promising role of PET-computed tomography, not only in the diagnosis of aortitis but also in monitoring disease activity, has been noted. Results of in-vitro studies on Janus kinase (JAK)/signal transducers and activators of transcription and mammalian target of rapamycin (mTOR) pathways, comparative studies with leflunomide as an induction therapy, and a long-term follow-up study with tocilizumab may contribute to the management of Takayasu arteritis. SUMMARY: An impressive number of studies have addressed aortitis in recent years. However, there still is a lack of robust data on causes, monitoring disease activity by imaging and biomarkers, and drugs providing steroid-free remission in noninfectious aortitis.

Comparison of Aortitis Versus Noninflammatory Aortic Aneurysms Among Patients Who Undergo Open Aortic Aneurysm Repair.

OBJECTIVE: Distinguishing aortitis-induced aneurysms from noninflammatory aortic aneurysms is difficult and often incidentally diagnosed on histologic examination after surgical repair. This study was undertaken to examine surgically diagnosed aortitis and identify patient characteristics and imaging findings associated with the disease. METHODS: In this case-control study, cases had newly diagnosed, biopsy-proven noninfectious aortitis after open thoracic aortic aneurysm surgical repair. Five controls were matched with cases for year of surgery and lacked significant inflammation on surgical pathology analysis. Data on comorbidities, demographic characteristics, and laboratory and imaging abnormalities prior to surgery were collected. Associations between exposures and outcomes were evaluated using conditional logistic regression. Backward stepwise logistic regression was used to determine factors independently associated with aortitis. Odds ratios (ORs) with 95%confidence intervals (95%CIs) were calculated. RESULTS: The study included 262 patients (43 patients with aortitis and 219 controls). Patients with aortitis were older at the time of surgery, predominantly female, and less likely to have a history of coronary artery disease (CAD). Multivariable analysis revealed that aortitis was independently associated with an older age at the time of surgery (OR 1.08 [95%CI 1.03-1.13], P < 0.01), female sex (OR 2.36 [95%CI 1.01-5.51], P = 0.04), absence of CAD (OR 6.92 [95%CI 2.14-22.34], P = 0.04), a larger aneurysm diameter (OR 1.74 [95%CI 1.02-2.98], P = 0.04), and arterial wall thickening on imaging (OR 56.93 [95%CI 4.31-752.33], P < 0.01). CONCLUSION: Among patients who undergo open surgical repair of an aortic aneurysm, elderly women with no history of CAD who have evidence of other aortic or arterial wall thickening on imaging are more likely to have histologic evidence of aortitis. Patients with these characteristics may benefit from further rheumatologic evaluation.

IgG4-aortitis among thoracic aortic aneurysms.

OBJECTIVE: The incidence of aortitis in patients with thoracic aortic diseases is not well established. The aim of this study was to analyse the frequency and clinical course of patients with aortitis in a surgical series. METHODS: 320 consecutive patients with ascending aorta/aortic arch aneurysm or acute aortic syndrome who underwent surgery from 2012 to 2017 in a single tertiary referral hospital were retrospectively reviewed. Epidemiological data, clinical course and variables related to diagnosis, treatment and follow-up were collected from patients with histologically proven aortitis. RESULTS: From 320 examined aortic samples, 279 (87.2%) thoracic aneurysms and 41 acute aortic syndromes (12.8%), 9 (2.8%) were aortitis: 3 cases of Takayasu's arteritis, 3 of IgG4-related aortitis, 2 of giant cell, and 1 classified as idiopathic. Median age at surgery was 53.4 (51-69.2) years and six cases were female. Seven patients presented with non-specific symptoms and the diagnosis was made at pathology. Surgery was elective in eight patients and emergent in one case of IgG4-related aortitis. 18F-fluorodeoxyglucose positron emission tomography/CT (18F-FDG PET/CT) was performed for disease extension study and as a monitoring technique during the follow-up of five patients, with just one case performed presurgically. All the patients with IgG4-related disease showed extrathoracic aortic involvement. There were no deaths, neither in-hospital nor during the 1.7 years of median follow-up. CONCLUSIONS: In surgically treated thoracic aorta pathology, the frequency of aortitis is low; IgG4-related disease is among the most common aetiologies with a frequency similar to other types of aortitis, such as Takayasu's and giant cell arteritis, and clinical manifestations are non-specific making presurgical diagnosis difficult. 18F-FDG PET/CT allows a better assessment of disease extension and therapeutic response. Surgery can be successfully performed and corticosteroid therapy ensures a good mid-term follow-up.

Clinical phenotypes of IgG4-related disease: an analysis of two international cross-sectional cohorts.

OBJECTIVE: IgG4-related disease (IgG4-RD) is a heterogeneous, multiorgan condition of unclear aetiology that can cause organ failure. Difficulty recognising IgG4-RD contributes to diagnostic delays. We sought to identify key IgG4-RD phenotypes. METHODS: We used two cross-sectional studies assembled by an international, multispecialty network of IgG4-RD specialists who submitted 765 cases to derive and replicate phenotypic groups. Phenotype groups of disease manifestations and key covariate distributions across the identified groups were measured using latent class analysis. RESULTS: In the derivation cohort (n=493), we identified four groups with distinct manifestations: Group 1 (31%), Pancreato-Hepato-Biliary disease; Group 2 (24%), Retroperitoneal Fibrosis and/or Aortitis; Group 3 (24%), Head and Neck-Limited disease and Group 4 (22%), classic Mikulicz syndrome with systemic involvement. We replicated the identification of four phenotype groups in the replication cohort. Compared with cases in Groups 1, 2 and 4, respectively, cases in Group 3 were more likely to be female (OR 11.60 (95% CI 5.39 to 24.98), 10.35 (95% CI 4.63 to 23.15) and 9.24 (95% CI 3.53 to 24.20)) and Asian (OR 6.68 (95% CI 2.82 to 15.79), 7.43 (95% CI 2.97 to 18.56) and 6.27 (95% CI 2.27 to 17.29)). Cases in Group 4 had a higher median serum IgG4 concentration (1170 mg/dL) compared with groups 1-3 (316, 178 and 445 mg/dL, respectively, p<0.001). CONCLUSION: We identified four distinctive IgG4-RD phenotypes according to organ involvement. Being Asian or female may predispose individuals to head and neck-limited disease. These phenotypes serve as a framework for identifying IgG4-RD and studying its aetiology and optimal treatment.

Incidence of Aortitis in Surgical Specimens of the Ascending Aorta Clinical Implications at Follow-Up.

The objectives of this study are to evaluate the incidence of aortitis on a surgical population, establish any relationship with systemic diseases, verify early and late surgical results and provide clinical and radiological follow-up to determine factors potentially predicting progression of the disease and influencing late outcome. From 2009 to 2017, 237 patients underwent elective operations on the ascending aorta. Segments of the excised tissues were routinely sent for histologic evaluation, providing adequate data in 178 (75%) for a clinical and pathologic correlation. Patients with aortitis (Group 1) (n = 26) were compared with 152 with atherosclerotic or degenerative disease (Group 2). Incidence of aortitis was 15%, being clinically isolated in 73%. In 24 patients (92%), a giant cell aortitis was found. Actuarial survival at 3 years is 88% in Group 1 and 98% in Group 2 and 74% and 98% at 5 years, respectively (P = 0.016). A control angio-computed tomography revealed an increased descending aorta diameter in 2 out of 14 late survivors. A positron emission tomography showed presence of arteritis in other vascular segments in 3 patients. Clinically isolated aortitis is extremely frequent in patients with inflammatory aortic disease. The diagnosis is often difficult and may be supported by routine pathologic evaluation of surgical explants and by multimodality imaging. The latter should be employed to allow adequate patient follow-up and to disclose potential recurrences in untreated aortic segments.

Impact of Medical Therapy on Late Morbidity and Mortality After Aortic Aneurysm Repair for Aortitis.

BACKGROUND: Patients with active aortitis who undergo repair of ascending aortic aneurysms have an increased risk of late reoperation and decreased late survival. We aimed to determine the reasons for these poor outcomes and the influence of medical management. METHODS: We reviewed records of 186 patients (median age 73.9 years; 120 women) with noninfectious aortitis after elective ascending aortic aneurysm repair (January 1955 through December 2012). Landmark analysis was used to compare outcomes in patients with isolated aortitis versus with systemic sequelae of aortitis along with outcomes of treatment with glucocorticoids. RESULTS: At 15 years, the overall mortality was 88.3%; at 10 years, the overall reoperation rate was 28.2%. Long-term mortality increased with older age at surgery (hazard ratio [HR] 1.62, 95% confidence interval [CI]: 1.25 to 2.11, p < 0.001), coronary artery disease (HR 1.94, 95% CI: 1.25 to 3.01, p = 0.003), peripheral vascular disease (HR 1.79, 95% CI: 1.09 to 2.94, p = 0.02), and preoperative suspicion of aortitis (HR 4.90, 95% CI: 1.96 to 12.26, p < 0.001). Increased reoperation rate was associated with coronary artery disease (HR 2.69, 95% CI: 1.17 to 6.17, p = 0.02) and peripheral vascular disease (HR 3.92, 95% CI: 1.71 to 8.94, p = 0.001). Among patients free of reoperation at 6 months, systemic sequelae of aortitis were found to be significant, with an unadjusted hazard ratio of 3.59 (95% CI: 1.40 to 9.18, p = 0.008). Treatment with glucocorticoids was not associated with subsequent mortality or reoperation. CONCLUSIONS: The development of systemic illness secondary to aortitis was associated with increased risk of late aortic reoperations. However, glucocorticoid treatment of noninfectious aortitis did not clearly influence survival or need for reoperation.

The effects of HIV and smoking on aortic and splenic inflammation.

OBJECTIVE: To determine the association of smoking and HIV status with tissue-specific inflammation measured by flurodeoxyglucose positron emission tomography (PET). DESIGN: A cross-sectional study. METHODS: We prospectively enrolled 55 HIV study participants on stable antiretroviral therapy and 19 age-matched HIV-uninfected controls without known cardiovascular disease. We measured aortic target-to-background ratio (TBR) and spleen standardized uptake values (SUV) 3-h post-FDG, and used regression models to examine the independent association of HIV and smoking status with PET variables. RESULTS: Overall, median (interquartile range) age was 50 (42-55) years; 81% were men and 54% were current smokers (median 0.5 packs/day, 25 pack-years]. Median CD4 of HIV study participants was 690 cells/ml and 88% had HIV-1 RNA less than 20 c/ml; 43% were on a protease inhibitor. In fully adjusted models, HIV was associated with 0.16 (95% confidence interval 0.04-0.27; P = 0.009) higher aortic TBR, whereas current smoking was marginally associated with a lower TBR [-0.11 (95% confidence interval -0.23 to 0.01); P = 0.07]. Spleen SUVmean was not associated with HIV or smoking, and there was no evidence for an HIVsmoking interaction for aortic or spleen models (all P > 0.1). Spleen SUVmean was positively associated with biomarkers of inflammation and coronary artery calcium, but adjustment for traditional cardiovascular disease risk factors attenuated these relationships. CONCLUSION: The FDG-PET study of HIV study participants suggests that HIV is associated with increased aortic inflammation independent of traditional risk factors, but smoking is not. Future studies should continue to explore the mechanistic roles of smoking and inflammation at various stages of clinical and subclinical atherosclerotic vascular disease in HIV.

Large-vessel vasculitis in human immunodeficiency virus-infected patients.

OBJECTIVE: The objective of this study was to describe large-vessel vasculitis (LVV) in patients with human immunodeficiency virus (HIV) infection. It is a retrospective single-center study conducted between 2000 and 2015 through a university hospital of 11 HIV-infected patients with LVV. METHODS: The characteristics and outcome of 11 HIV-infected patients with LVV (7 patients fulfilled international criteria for Takayasu arteritis, 5 patients had histologic findings of vasculitis, and 5 patients had imaging features of aortitis) were analyzed and compared with those of 82 patients with LVV but without HIV infection. RESULTS: Concerning the HIV-infected patients with LVV (n = 11), the mean age was 40 years (range, 36-56 years), and 55% of patients were female. At diagnosis of LLV, the mean initial CD4 cell count was 455 cells/mm3 (range, 166-837 cells/mm3), and the median HIV viral load was 9241 copies. Vascular lesions were located in the aorta (n = 7), in supra-aortic trunks (n = 7), and in digestive arteries (n = 3). Inflammatory aorta infiltrates showed a strong expression of interferon-γ and interleukin 6. In HIV-negative LVV patients (n = 82), the median age was 42 years, and 88% of the patients were women. Thirty patients had an inflammatory syndrome. Seventy patients had been treated with glucocorticosteroids and 57 with immunosuppressive treatments. Compared with their negative counterparts, HIV-positive patients with LVV were more frequently male (P = .014), had more vascular complications (ie, Ishikawa score; P = .017), and had more frequent revascularization (P = .047). After a mean follow-up of 96 months, four relapses of vasculitis were reported, and one patient died. Regardless of the HIV virologic response, antiretroviral therapy improved LVV in only one case. CONCLUSIONS: LVV in HIV-infected patients is a rare and severe entity.

Aortic involvement in relapsing polychondritis.

OBJECTIVE: To assess prevalence of aortic involvement in relapsing polychondritis (RP) patients; to evaluate clinical features and long-term outcome of RP patients exhibiting aortitis, aortic ectasia and/or aneurysm. METHODS: One hundred and seventy-two RP patients underwent aortic computed tomography (CT)-scan; they were seen in 3 medical centers. RESULTS: Eleven patients (6.4%) had aortic involvement, occurring within a median time of 2 years after RP diagnosis. CT-scan showed isolated aortitis (n=2); the 9 other patients exhibited: aortitis and aortic aneurysm (n=2) or ectasia (n=1), isolated aortic aneurysm (n=4) or ectasia (n=2); aortic localizations were as follows: thoracic (n=6), abdominal (n=2), thoracic and abdominal (n=4) aorta. Patients exhibited: resolution (n=3) improvement (n=3), stabilization (n=4) or deterioration (n=1) of aortic localization. Five patients experienced recurrence of aortic localization; one patient died of aortic abdominal aneurysm rupture. Predictive factors of death related to aortic complications were: aortitis on CT-scan, higher median levels of erythrocyte sedimentation rate. Predictive parameters of aortic relapses were: aortitis on CT-scan and involvement of the abdominal aorta. CONCLUSIONS: This study underlines that aortic involvement is severe in RP. Furthermore, we suggest that RP patients exhibiting poor prognostic factors, including panaortitis and higher values of ESR, may require more aggressive therapy.

Clinical features of IgG4-related periaortitis/periarteritis based on the analysis of 179 patients with IgG4-related disease: a case-control study.

BACKGROUND: Immunoglobulin G4-related disease (IgG4-RD) is a newly recognized systemic condition characterized by high serum immunoglobulin G4 (IgG4) concentration and IgG4-bearing plasma cell infiltration in affected organs. Although it has become evident that IgG4-RD also involves the systemic aortic/arterial system, the precise details of this condition remain unclear. The present study sought to clarify the clinical features of IgG4-related periaortitis/periarteritis. METHODS: Among 223 patients with IgG4-RD, 179 (131 male, median onset age 67 years) were recruited for this study. Periaortitis/periarteritis was defined as vessel wall thickness with circumferential enhancement on contrast-enhanced computed tomography. RESULTS: Periaortitis/periarteritis was identified in 65 (36.3%; 53 male) of 179 IgG-RD patients. The distribution of IgG4-related periaortitis/periarteritis could be broadly classified into five types, with the most prevalent Type 2 (44.6%) being localized at the infra-renal artery portion of the abdominal aorta and continuing to the iliac arteries. The infra-renal artery region of the abdominal aorta was most frequently involved (>80%) among IgG4-related periaortitis/periarteritis cases. Comparisons of clinical parameters between IgG4-RD patients with and without periaortitis/periarteritis revealed significantly higher propensities for older IgG4-RD onset age and highly active disease state featuring elevated serum IgG, IgG4, circulating immune complex, and soluble interleukin-2 receptor. All patients showed improvement of wall thickening after steroid therapy, although nine patients (20.9%) exhibited worsening of luminal dilatation. The main risk factor for this manifestation was prior luminal dilatation according to multivariate analysis. CONCLUSION: IgG4-related periaortitis/periarteritis predominantly occurred at the infra-renal artery portion of the abdominal aorta, affected older IgG4-RD onset patients, and was prevalent in highly active disease states. As reported previously, the main risk factor for worsening luminal dilation after corticosteroid therapy was the existence of luminal dilation beforehand.