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A 54-year-old female presented with recurrent abdominal pain and new onset chest pain. Chest computed-tomography angiogram detected a thoracic aortic aneurysm with suspected Type A intramural hematoma (IMH) versus aortitis. Initially, conservative management was pursued while awaiting a definitive diagnosis. Differential workup was negative, while additional imaging modalities favored IMH, prompting expedited surgical intervention. During ascending aortic and hemiarch replacement, severe aortitis was unexpectedly discovered without evidence of IMH. Histopathological examination of the aortic specimens identified transmural aortic inflammation with lymphoplasmacytic infiltrate and irregular fibrosis. Numerous IgG4-positive plasma cells were present with IgG4/IgG ratio of 40-50% suggesting IgG4-related disease (IgG4-RD). Subsequent analysis revealed B cells positive for clonal IgH gene rearrangement, and bone marrow biopsy then revealed the same clonal B cells. She was ultimately diagnosed with CLL, the most common phenotype of monoclonal B-cell lymphocytosis, thought to account for the IgG4-predominant plasma cells causing aortitis. Although rare, this case highlights the importance of considering IgG4-related disease (IgG4-RD) as a cause of aortitis when assessing symptomatic patients with aortic pathologies, emphasizing the complexities involved in diagnosing due to a variety of imaging presentation, differentiating, and managing large-vessel vasculitides. Moreover, it underscores the importance of Multidisciplinary Aortic Team care and the use of multiple diagnostic modalities in evaluating ambiguous aortic pathologies., (© 2024. The Author(s).)

Noninfectious aortitis is occasionally detected incidentally, either on imaging or on histopathologic review after open thoracic aortic surgery. It can present as a clinically asymptomatic, seemingly focal lesion, as diffuse inflammation throughout several aortic segments but sparing the branch vessels, or as a manifestation of a widespread systemic condition. Treatment differs based on etiology, so once identified, all patients with aortitis need a thorough evaluation, laboratory tests, complete large-vessel imaging, and a referral to a vasculitis expert. All patients with aortitis are at high risk of future vascular complications and should be followed with serial clinical evaluations and imaging., (Copyright © 2024 The Cleveland Clinic Foundation. All Rights Reserved.)

Background: IgG4-related disease (IgG4-RD) is a rare, systemic immune-mediated fibro-inflammatory condition with an unclear etiology and pathophysiology, potentially affecting multiple organs. It presents with common clinical, radiological, and serological characteristics. This study aims to compare the latest two IgG4-RD classification and diagnostic criteria: Umehara-Okazaki 2011 and ACR/EULAR 2019., Material and Methods: In a retrospective cross-sectional study conducted across two centers from January 2010 to July 2023, we included patients suspected of having IgG4-RD from various hospital departments. Patients finally diagnosed with other pathologies were excluded. The remaining suspected IgG4-RD cases were evaluated using both Umehara-Okazaki 2011 and ACR/EULAR 2019 criteria., Results: Out of 34 patients with a clinical diagnosis of IgG4-RD, the Umehara-Okazaki 2011 classified 20 patients: 5 as definitive, 7 as probable, and 8 as possible cases. Applying the ACR/EULAR 2019 criteria to the same cohort resulted in the diagnosis of 9 patients. Notably, retroperitoneal fibrosis and aortitis were the most prevalent form of presentation, accounting for 25% and 22.2% of cases classified under the 2011 and 2019 criteria, respectively., Discussion: The more recent and stringent ACR/EULAR 2019 criteria focus on histopathology, various forms of presentation, and analytical data, allow for a more accurate classification of patients., (Copyright © 2024 Elsevier España, S.L.U. All rights reserved.)

Infective native arterial aneurysms and inflammatory aortic aneurysms are rare but morbid pathologies seen by vascular surgeons in the emergency setting. Presentation is not always clear, and a full workup must be obtained before adopting a management strategy. Treatment is multidisciplinary and is tailored to every case based on workup findings. Imaging with computed tomography, magnetic resonance, or with fluorodeoxyglucose-positron emission tomography aids in diagnosis and in monitoring response to treatment. Open surgery is traditionally performed for definitive management. Endovascular surgery may offer an alternative treatment in select cases with acceptable outcomes. Neither technique has been proven to be superior to the other. Physicians should consider patient's anatomy, comorbidities, life expectancy, and goals of care before selecting an approach. Long-term pharmacological treatment, with antibiotics in case of infective aneurysms and immunosuppressants in case of inflammatory aneurysms, is usually required and should be managed in collaboration with infectious disease specialists and rheumatologists., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Infectious aortitis is a rare disease process which can be of fungal, viral or bacterial aetiology. This disease process is often incidentally found during concomitant infectious processes, likely due to haematogenous spread. Common sources are from cardiac, genitourinary and gastroenterologic sources. CT imaging of the aorta is essential in identifying physiological changes-wall thickness changes, ectasia and stenosis. We present a case of a female in her early 60s with a medical history of cardiomyopathy with heart failure and reduced ejection fraction, who was initially admitted for acute cholecystitis complicated by the development of gallstone pancreatitis. Imaging evaluation incidentally noted findings consistent with aortitis with a penetrating ulcer, and blood cultures were positive for Staphylococcus aureus bacteraemia, confirming her diagnosis of infectious aortitis. She was started on intravenous antibiotics, required preoperative nutritional optimisation, and subsequently underwent an open aortic resection and aortoiliac reconstruction with rifampin-soaked Dacron graft., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

Syphilitic aortitis is a rare disease caused by Treponema pallidum affecting the aorta and leading to inflammation. Syphilitic aortitis is one of the causes of aortic aneurysms. This article presents surgical treatment of a patient with syphilitic aortitis and thoracic aortic aneurysm. This clinical case confirms the difficulties of surgical treatment.

Aortitis is a life-threatening, manifestation of chronic Q fever. We report a series of 5 patients with Q fever aortitis who have presented to our hospital in tropical Australia since 2019. All diagnoses were confirmed with polymerase chain reaction (PCR) testing of aortic tissue. Only one had a previous diagnosis of acute Q fever, and none had classical high-risk exposures that might increase clinical suspicion for the infection. All patients underwent surgery: one died and 3 had significant complications. Q fever aortitis may be underdiagnosed; clinicians should consider testing for Coxiella burnetii in people with aortic pathology in endemic areas., (© 2023. The Author(s).)

BACKGROUND Heart failure is caused by coronary artery disease, valvular disease, and arrhythmias and is highly treatable with recent technology. However, the incidence of syphilis is increasing worldwide. This case report describes tertiary cardiovascular syphilis, accompanied by aortic regurgitation, syphilitic aortitis complicated by thrombus of the ascending aorta, and coronary artery occlusion, requiring percutaneous coronary artery intervention. CASE REPORT A 51-year-old Japanese man with no significant medical history was admitted to the hospital for worsening shortness of breath on exertion. On physical examination, there was no edema in either lower leg. Chest X-rays showed an enlarged heart and pulmonary congestion, and echocardiography showed a left ventricular ejection fraction of 18%, with full circumferential wall motion impairment. Heart failure was diagnosed, and the patient was found to have severe coronary artery disease and aortic regurgitation. He underwent percutaneous coronary intervention (PCI) for his coronary artery occlusion and was treated with medications for heart failure. Two months later, his condition improved, and PCI was performed for the revascularization of the remaining coronary artery. After PCI was completed, the patient was evaluated for vasculitis. The aortic wall lesion was likely a result of non-active syphilitic aortitis, and the results of serological tests of syphilis were positive. Therefore, we concluded that the diagnosis was cardiovascular syphilis. CONCLUSIONS This case report has highlighted the need for clinicians to be aware of the cardiovascular findings in syphilis, including syphilitic aortitis, particularly at this time, when the global incidence of syphilis is increasing.

Background: Salmonella spp. cause infectious aortitis through the hematogenous spread of an intestinal Salmonella infection. Salmonella aortitis can result in extensive tissue damage in the aorta leading to complications including dissection, abscess formation, pseudoaneurysms, and rupture, which require early diagnosis and treatment with both surgery and antibiotic therapy., Case Presentation: We report a case of Salmonella aortitis complicated by Stanford type A aortic dissection. A 62-year-old man with a history of heroin use presented with chest pain, epigastric pain and vomiting. The computed tomography scan showed Stanford type A aortic dissection without malperfusion. At the time of surgery, an aortic dissection with purulent fluid and contained rupture was noted in the ascending aorta. Fluid culture was consistent with Salmonella. A composite valve-graft conduit aortic root replacement with ascending aorta and hemiarch replacement was performed. The patient recovered well and was discharged on long-term antibiotics., Conclusions: This rare case of a Stanford type A aortic dissection with contained rupture due to Salmonella aortitis was successfully treated with emergent surgery and antibiotic therapy., (© 2023. The Author(s).)

BACKGROUND Acute aortic insufficiency can be secondary to multiple conditions, including infective endocarditis, aortic root pathologies (eg, dissection, aortitis), or traumatic injury. Aortitis involves a broad spectrum of disorders characterized by inflammatory changes in the aortic wall. This pathology can be subsequently classified depending on its etiology into inflammatory and infectious causes. Large-vessel vasculitis (giant-cell arteritis, Takayasu arteritis, and IgG4-related vasculitis) is the most common non-infectious causes of aortitis. Giant-cell aortitis usually lacks the classic clinical findings of giant-cell arteritis such as headache, visual symptoms, or jaw claudication, which can be a diagnostic challenge. However, clinicians should have a high index of suspicion, since this pathology can evolve into potentially life-threatening conditions, including aortic aneurysm, aortic wall rupture, and aortic acute dissection. CASE REPORT We present a case of a 76-year-old woman who presented to the Emergency Department (ED) with shortness of breath associated with orthopnea, paroxysmal nocturnal dyspnea, and mild productive cough with white sputum. A transthoracic echocardiogram demonstrated reduced left ventricular ejection fraction, dilated left ventricle, and severe aortic insufficiency. Cardiac catheterization revealed mild non-obstructive coronary arteries and severe aortic regurgitation. The surgical pathology report of the portion of the aorta was consistent with giant-cell aortitis. CONCLUSIONS In this article, we present a case of giant-cell aortitis as an unusual etiology of acute aortic insufficiency, which is most probably under-detected in clinical practice. In addition to describing the case, we aim to highlight the importance of proper ascending aorta evaluation in patients presenting with new-onset aortic regurgitation and heart failure to prevent associated morbidity and mortality.

Aortic involvement in immunoglobulin G4-related disease (IgG4-RD) is extremely rare and is often overlooked during the aortitis work-up. IgG4-related aortitis differs from non-IgG4-related aortitis in its histopathological features, site of involvement, laboratory markers, and treatment options. The histopathological examination of the vessel walls characteristically reveals adventitial thickening with intimal sparing, typically affecting the infrarenal abdominal aorta. In addition, inadequate knowledge about the disease often leads to delayed or missed diagnosis and undermanagement of a potentially treatable condition. Hence, in this paper, we review the unique clinical manifestations, laboratory markers, diagnostic features, current treatment strategies, and novel experimental therapeutic options in the management of IgG4-related aortitis.

Background: Prognosis data on giant-cell arteritis (GCA)-associated aortitis are scarce and heterogeneous. The aim of this study was to compare the relapses of patients with GCA-associated aortitis according to the presence of aortitis on CT-angiography (CTA) and/or on FDG-PET/CT., Methods: This multicenter study included GCA patients with aortitis at diagnosis; each case underwent both CTA and FDG-PET/CT at diagnosis. A centralized review of image was performed and identified patients with both CTA and FDG-PET/CT positive for aortitis (Ao-CTA+/PET+); patients with positive FDG-PET/CT but negative CTA for aortitis (Ao-CTA-/PET+), and patients solely positive on CTA., Results: Eighty-two patients were included with 62 (77%) of female sex. Mean age was 67±8 years; 64 patients (78%) were in the Ao-CTA+/PET+ group; 17 (22%) in the Ao-CTA-/PET+ group and 1 had aortitis only on CTA. Overall, 51 (62%) patients had at least one relapse during follow-up: 45/64 (70%) in the Ao-CTA+/PET+ group and 5/17 (29%) in the Ao-CTA-/PET+ group (log rank, p = 0.019). In multivariate analysis, aortitis on CTA (Hazard Ratio 2.90, p = 0.03) was associated with an increased risk of relapse., Conclusion: Positivity of both CTA and FDG-PET/CT for GCA-related aortitis was associated with an increased risk of relapse. Aortic wall thickening on CTA was a risk factor of relapse compared with isolated aortic wall FDG uptake., Competing Interests: Declaration of Competing Interest Authors have no conflict of interest for this study., (Copyright © 2023 Elsevier Inc. All rights reserved.)

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., Competing Interests: Potential conflicts of interest. P. R. reports payment or honoraria from Celgene, Novartis, and AdLiva and support for attending meetings and/or travel from Janssen-Cilag, Amgen, Roche, Takeda, Mylan, and Celgene. All other authors report no potential conflicts. All authors have submitted the ICMJE Form for Disclosure of Potential Conflicts of Interest. Conflicts that the editors consider relevant to the content of the manuscript have been disclosed., (© The Author(s) 2022. Published by Oxford University Press on behalf of Infectious Diseases Society of America. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Background: SARS-CoV-2 may trigger both vasculitis and arrhythmias as part of a multisystem inflammatory syndrome described in children as well as in adults following COVID-19 infection with only minor respiratory symptoms. The syndrome denotes a severe dysfunction of one or more extra-pulmonary organ systems, with symptom onset approximately 2-5 weeks after the COVID-19 infection. In the present case, a seemingly intractable ventricular tachycardia preceded by SARS-CoV2 infection was only managed following the diagnosis and management of aortitis., Case Presentation: A 69-year-old woman was hospitalized due to syncope, following a mild COVID-19 infection. She presented with paroxysmal atrial fibrillation and intermittent ventricular tachycardia interpreted as a septum-triggered bundle branch reentry ventricular tachycardia, unaffected by amiodaron, lidocaine and adenosine. A CT-scan revealed inflammation of the aortic arch, extending into the aortic root. In the following days, the tachycardia progressed to ventricular storm with intermittent third-degree AV block. A temporary pacemaker was implanted, and radiofrequency ablation was performed to both sides of the ventricular septum after which the ventricular tachycardia was non-inducible. Following supplemental prednisolone treatment, cardiac symptoms and arrythmia subsided, but recurred after tapering. Long-term prednisolone treatment was therefore initiated with no relapse in the following 14 months., Conclusion: We present a rare case of aortitis complicated with life-threatening ventricular tachycardia presided by Covid-19 infection without major respiratory symptoms. Given a known normal AV conduction prior to the COVID-19 infection, it seems likely that the ensuing aortitis in turn affected the septal myocardium, enabling the reentry tachycardia. Generally, bundle branch reentry tachycardia is best treated with radiofrequency ablation, but if it is due to aortitis with myocardial affection, long-term anti-inflammatory treatment is mandatory to prevent relapse and assure arrhythmia control. Our case highlights importance to recognize the existence of the multisystem inflammatory syndrome in adults (MIS-A) following COVID-19 infection in patients with alarming cardiovascular symptoms. The case shows that the early use of an CT-scan was crucial for both proper diagnosis and treatment option., (© 2023. The Author(s).)

An 86-year-old man who underwent endovascular aortic repair for impending rupture of an abdominal aortic aneurysm a year ago presented to our hospital because of fatigue and black stools. Multiple bacterial specimens were detected in blood cultures, and computed tomography following oral administration of gastrografin demonstrated gastrografin in the abdominal aorta. The diagnosis of aortic duodenal fistula was confirmed and emergency abdominal aortic replacement was performed. The pathological findings of the aorta included a large number of immunoglobulin G4 (IgG4)-positive plasma cells infiltrating all layers of the aortic wall, with particularly marked thickening of the adventitia. The serum IgG4 level was 241 mg/dl and IgG4-related periaortitis was diagnosed. Aortoduodenal fistula is a rare but fatal complication of IgG4-related periaortitis. Patients should be followed carefully after endovascular aortic repair for inflammatory abdominal aortic aneurysms., (© Japan College of Rheumatology 2022. Published by Oxford University Press. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Introduction: Giant cell arteritis (GCA) or Horton’s disease is a segmental and focal inflammation of large and medium-sized arteries mostly seen in patients of 50 years and older. There is also a peak frequency in individuals between the ages of 70 and 80. However, clinical data is scarce in this age group and especially in patients over 80., Methods: A retrospective study comprised of patients diagnosed with Horton’s arteritis between 2012 and 2017, according to the American Society of Rheumatology, was conducted at Reims University Hospital. Patients were assigned to two groups according to age (≤ 75 and < 75) in order to evaluate and compare the impact of age on diagnosis, treatment and prognosis., Results: A total of 67 patients were studied. The mean age upon diagnosis was 75,85 ±8.5 years; 36 patients (53.7%) 75 years or younger and 31 patients older than 75. There was a female predominance (43 patients), 22 patients aged 75 years or younger and 21 older than 75. The mean follow up duration was 43.02 months in patients aged 75 years or younger and 30.99 in patients older than 75. This represents a difference of more than one year in terms of follow up, but is not statistically significant (p = 0.620). Eleven patients (16.4%) died during follow up: 5 patients (13.9%) aged 75 years or younger and 6 patients (19.4%) older than 75 (p = 0.547). Aortitis was significantly less seen in patients older than 75 (p = 0.0410)., Conclusion: Our study showed no significant difference in either age group. However, aortitis was less seen in patients older than 75 years. Patients aged 75 or younger seemed more prone to relapses, but their follow up periods were shorter.

Competing Interests: The authors declare no conflict of interest.

Objectives: Chronic periaortitis (CP) is a less known but more frequently diagnosed fibro-inflammatory disorder, but we know little about it and data regarding follow-up and outcome are still very limited. This study aims to identify the clinicopathologic, laboratory, and radiologic features, as well as outcomes of CP patients., Methods: Patients with CP from HUVAC database were included in the study. CP was diagnosed based on compatible imaging findings and histopathological evaluation (if available), in addition to clinical findings. Demographics, laboratory, clinical, and imaging data were retrospectively reviewed from medical records., Results: A total of 51 (male/female:37/14) patients were included in the study. Median (IQR) age was 63 (53-69) years and follow-up duration was 40 (4-60) months. 32 of the patients were IgG4-related CP. The most common form of CP in our cohort was idiopathic retroperitoneal fibrosis (82%), followed by inflammatory abdominal aortic aneurysms (12%) and peri-aneurysmal retroperitoneal fibrosis (8%). 8 (15.6%) patients had thoracic periaortitis and 16 (31.6%) venous involvement. Cyclophosphamide (CYC) combined with steroids was the most preferred treatment modality (43%), followed by rituximab (RTX) (31.3%). Follow-up imaging was done after a median (IQR) of 7(3-11) months, 30% of the patients were stable and 64.1% showed regression. A total of 18 (35.2%) had been taken off therapy at the last visit., Conclusions: Idiopathic retroperitoneal fibrosis was the most frequent presentation, whereas 15.6% of patients had thoracic involvement. Venous involvement was also not uncommon. Optimal time for follow-up imaging was determined as 6-9 months. Steroids along with CYC/RTX had a favourable outcome in the treatment of these patients.

Introduction: Lymphoplasmacytic thoracic aortitis is still underrecognized cause of thoracic aortic aneurysm., Case Report: We herein report a case of symptomatic inflammatory ascending aortic aneurysm and pericarditis diagnosed on CT scan as acute type A intramural hematoma. Patient underwent emergency supracoronary ascending aorta replacement with a prosthetic graft. Marked inflammatory thickening of aneurysm wall was the intraoperative finding. Histopathologic examination of the excised aortic tissue showed inflammation with lymphoplasmacytic pattern of aortitis., Conclusion: This case demonstrates that lymphoplasmacytic aortitis may mimic symptoms and radiological findings of intramural hematoma.

Competing Interests: The authors declare no conflict of interest.

Competing Interests: The authors declare no conflict of interest.

Competing Interests: The authors declare no conflict of interest.

We report an unusual case of a 35-year-old Ivorian migrant with an abdominal mass and medical history relevant for human immunodeficiency virus-2 positivity with a CD4/CD8 ratio of 0.63; Mantoux and lymphocyte stimulation tests (QuantiFERON) were positive. 3D-CT images revealed a voluminous non-homogeneous retroperitoneal mass in the right abdominal region presenting no significant contrast impregnation. Thoraco-abdominal aorta presented diffuse-altered morphology with multiple ectasias throughout its course and an aneurysm at the level of the subrenal tract. The patient underwent vascular surgery. Mycobacterium tuberculosis complex was detected by polymerase chain reaction performed on intraoperative tissue specimens. Postoperative course was uneventful. After surgery, 3D-CT images showed no signs of malfunction of the prosthesis. At last, at 6-month follow-up, the patient was well. Cross-sectional imaging techniques, such as contrasted-CT, are essential and allow for making diagnosis, assessing disease activity, and evaluating post-treatment condition. 3D reconstruction permits an appropriate patient care by means of an excellent visualization and staging of the disease process.

IgG4-related disease is an immune-mediated fibro-inflammatory disorder with multisystemic involvement. Aortitis and peri-aortitis are the most common cardiovascular manifestations of the disease. We present the case of a 65-year-old man with symptomatic severe aortic stenosis and concomitant IgG4 aortitis. The diagnosis was confirmed by IgG4 serum levels, positive positron emission computed tomography (PET-CT) scans, and pathology from mediastinal dissection. Surgical aortic valve replacement (SAVR) was unfeasible owing to extensive mediastinal fibrosis, and transcatheter aortic valve replacement (TAVR) was successfully performed. As ascending aorta access for SAVR in IgG4 aortitis with long-run fibrosis entails a high risk of mortality, TAVR could be considered in certain suitable patients., (Copyright © 2021 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.)

Primary aortitis (PA) secondary to Listeria monocytogenes is extremely rare with only a few cases reported in the literature. Presently, there is no consensus concerning the best treatment when no complications are found in the thoracic computed tomography (CT) imaging. This report illustrates the clinical presentation and favorable clinical course of a rare case of PA secondary to Listeria monocytogenes in an 82-year-old diabetic woman, successfully treated with conservative management with 18 months of follow up. Included in this article, we additionally present a review of the literature of this uncommon etiology of infectious aortitis.

Broadly defined, aortitis refers to inflammation of the aorta and incorporates both infectious and non-infectious aetiologies. As advanced imaging modalities are increasingly incorporated into clinical practice, the phenotypic spectrum associated with aortitis has widened. The primary large vessel vasculitides, giant cell arteritis and Takayasu arteritis, are the most common causes of non-infectious aortitis. Aortitis without systemic disease or involvement of other vascular territories is classified as clinically isolated aortitis. Periaortitis, where inflammation spreads beyond the aortic wall, is an important disease subset with a distinct group of aetiologies. Infectious aortitis can involve bacterial, viral or fungal pathogens and, while uncommon, can be devastating. Importantly, optimal management strategies and patient outcomes differ between aortitis subgroups highlighting the need for a thorough diagnostic workup. Monitoring disease activity over time is also challenging as normal inflammatory markers do not exclude significant vascular inflammation, particularly after starting treatment. Additional areas of unmet clinical need include clear disease classifications and improved short-term and long-term management strategies. Some of these calls are now being answered, particularly with regard to large vessel vasculitis where our understanding has advanced significantly in recent years. Work extrapolated from temporal artery histology has paved the way for targeted biological agents and, although glucocorticoids remain central to the management of non-infectious aortitis, these may allow reduced glucocorticoid reliance. Future work should seek to clarify disease definitions, improve diagnostic pathways and ultimately allow a more stratified approach to patient management., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2021. No commercial re-use. See rights and permissions. Published by BMJ.)

Objectives: The 2019 ACR/EULAR classification criteria for IgG4-related disease (IgG4-RD) have exclusion criteria including positive disease-specific autoantibodies, and these have been documented to have a high specificity. This study aimed to further validate these criteria as well as identify characteristics of patients showing false-negative results., Methods: We retrospectively analysed 162 IgG4-RD patients and 130 mimickers. The sensitivity, specificity and fulfilment rates for each criterion were calculated, and intergroup comparisons were performed to characterize the false-negative cases., Results: Both the IgG4-RD patients and mimickers were aged ≥65 years with male predominance. The final diagnoses of mimickers were mainly malignancy, vasculitis, sarcoidosis and aneurysm. The classification criteria had a sensitivity of 72.8% and specificity of 100%. Of the 44 false-negative cases, one did not fulfil the entry criteria, 20 fulfilled one exclusion criterion and 27 did not achieve sufficient inclusion criteria scores. The false-negative cases had fewer affected organs, lower serum IgG4 levels, and were less likely to have received biopsies than the true-positive cases. Notably, positive disease-specific autoantibodies were the most common exclusion criterion fulfilled in 18 patients, only two of whom were diagnosed with a specific autoimmune disease complicated by IgG4-RD. In addition, compared with the true-positive cases, the 18 had comparable serum IgG4 levels, number of affected organs, and histopathology and immunostaining scores despite higher serum IgG and CRP levels., Conclusions: The ACR/EULAR classification criteria for IgG4-RD have an excellent diagnostic specificity in daily clinical practice. Positive disease-specific autoantibodies may have limited clinical significance for the diagnosis of IgG4-RD., (© The Author(s) 2020. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

IgG4 aortitis is a recently recognized entity that can have clinical and imaging features that mimic acute aortic syndrome. Therefore, it is imperative for radiologists to be aware of how to potentially differentiate the two. Although this entity has been previously described via case reports and meta-analysis in the context of inflammatory abdominal aortic aneurysm, very few cases of ascending aortic involvement have been reported. In this case report, we present a case of a 60-year-old female transferred from another facility for an initial diagnosis of intramural hematoma of the ascending aorta and later found to have IgG4 aortitis post aortic root repair. This is a histologically confirmed case of multi-segmented IgG4 aortitis with rare involvement of both ascending and infra-renal aorta. We will briefly discuss the pathophysiology of IgG4 aortitis, along with review of literature., (Copyright Journal of Radiology Case Reports.)

A 24-year-old woman referred to our radiology department with the diagnosis of new positive blood culture brucellosis and severe aortic regurgitation for evaluation of periaortic thickening. Cardiac magnetic resonance imaging elegantly revealed the presence of active aortitis, most probably caused by brucellosis., (© 2021 Wiley Periodicals LLC.)

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.

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., (Copyright© 2021, International Institute of Anticancer Research (Dr. George J. Delinasios), All rights reserved.)

Aortic involvement is relatively common in the context of IgG4-related disease (IgG4-RD). It includes IgG4-aortitis, and IgG4-(chronic) periaortitis (IgG4-CP). The latter overlaps with IgG4-retroperitoneal fibrosis (IgG4-RPF). Aortic wall thickening which characterizes these entities along with the presence of periaortic tissue in IgG4-CP, are often accompanied by aortic aneurysms, which belong to the group of the so-called inflammatory aneurysms. Both the thoracic and abdominal aorta can be affected. Aortitis appears to involve more often the former, while the opposite is the case for IgG4-CP. There is a lack of definitions and different classification criteria have been used to describe these entities. This report provides an overview on the current evidence of aortic involvement in IgG4-RD. It discusses the clinical, epidemiologic, serologic and histopathologic characteristics, as well as the imaging techniques used for their diagnosis and the therapeutic options and treatment outcomes. The differential diagnosis and underlying pathogenetic mechanisms are also discussed., (Copyright © 2020 Elsevier B.V. All rights reserved.)

Aortitis includes conditions with infectious or non-infectious etiology, characterized by inflammatory changes in one or more layers in aortic wall. Age at onset, geographic predilections, distribution and pattern of involvement in aorta, its branches and pulmonary arteries, and systemic associations provide a clue to etiology. Clinical presentations are often non-specific. An integrated approach including clinical, laboratory and imaging assessment is essential to confirm diagnosis and plan treatment. Assessment of disease activity is the key as it influences timing and outcome of treatment. Markers of activity include clinical, laboratory and imaging. Medical management remains the first-line therapy. Revascularization is indicated in the presence of hemodynamically significant stenosis and inactive disease. In the presence of flash pulmonary edema, left ventricular dysfunction or hypertensive encephalopathy, revascularization is performed irrespective of disease activity. Endovascular management is favored over surgery due to its high success and low restenosis rates. Symptomatic aneurysmal disease is usually managed by surgery.

In the present case we report on a 51-year-old patient diagnosed with Cogan syndrome. This vasculitis of variable vessel size is a rare disease that poses a major challenge for the correct diagnostics and therapy. In the classic setting, it comprises a triad of non-syphilitic interstitial keratitis as well as hearing loss with vestibular dysfunction. A vascultis-related aortitis, an uncertain, more likely degenerative structure in combination with strongly elevated inflammation parameters was misinterpreted as infective endocarditis for a long time and treated with anti-infective medications. After diagnosis the patient recovered following treatment with high-dose steroids and in the further course cyclophosphamide and tumor necrosis factor‑α blockers.

Large vessel involvement in giant cell arteritis has long been described, although its right frequency and potential prognostic value have only been highlighted for two decades. Large vessel involvement not only is associated with a high incidence of late aortic aneurysms, but also might cause greater resistance to glucocorticoids and longer treatment duration, as well as worse late cardiovascular outcomes. These data were brought to our attention, thanks to substantial progress recently made in large vessel imaging. This relies on four single, often complementary, approaches of varying availability: colour Doppler ultrasound, contrast-enhanced computed tomography with angiography and, magnetic resonance imaging, which all demonstrate homogeneous circumferential wall thickening and describe structural changes; 18F-fluorodeoxyglucose positron emission tomography-computed tomography (PET/CT), which depicts wall inflammation and assesses many vascular territories in the same examination. In addition, integrated head-and-neck PET/CT can accurately and reliably diagnose cranial arteritis. All four procedures exhibit high diagnostic performance for a large vessel arteritis diagnosis so that the choice is left to the physician, depending on local practices and accessibility; the most important is to carry out the chosen modality without delay to avoid false or equivocal results, due to early vascular oedema changes as a result of high dose glucocorticoid treatment. Yet, ultrasound study of the superficial cranial and subclavian/axillary arteries remains a first line assessment aimed at strengthening and expediting the clinical diagnosis as well as raising suspicion of large-vessel involvement. In treated patients, vascular imaging results are poorly correlated with clinical-biological controlled disease so that it is strongly recommended not to renew imaging studies unless a large vessel relapse or complication is suspected. On the other hand, a structural monitoring of aorta following giant cell arteritis is mandatory, but uncertainties remain regarding the best procedural approach, timing of first control and spacing between controls. Individuals at greater risk of developing aortic complication, e.g. those with classic risk factors for aneurysm and/or visualised aortitis, should be monitored more closely., (Copyright © 2020. Published by Elsevier Masson SAS.)

A 41-year-old female captive gorilla with progressive weight loss and hydrothorax of unknown origin was euthanized and submitted for necropsy. The ascending aorta showed intimal aortic thickenings, consistent with so called 'tree bark' changes. Microscopic examination revealed a non-infectious, necrotizing and granulomatous aortitis with no evidence of systemic vasculitis or infectious disease elsewhere in the body. While rare, large vessel vasculitides should be considered as a differential diagnosis in gorillas presenting with progressive non-specific signs and vascular intimal changes., (Copyright © 2020 Elsevier Ltd. All rights reserved.)

Lupus aortitis is a rare and potentially life-threatening disorder. Previous studies have reported the utility of high-dose systemic glucocorticoids or surgery as the treatment, although there have been no related controlled trials. We herein report a 49-year-old woman with a 35-year history of systemic lupus erythematosus who was diagnosed with aortitis. Her symptoms and laboratory and imaging abnormalities rapidly resolved upon the administration of moderate-dose glucocorticoids. We subsequently performed a literature review of similar cases to identify the appropriate treatment and discuss these cases. A study of further cases will be needed to identify the characteristics of patients who would benefit from moderate-dose glucocorticoid therapy.

IgG4-related disease (IgG4-RD) is a fibro-inflammatory condition characterized by increased serum IgG4 level, infiltration of lymphocytes and IgG4-positive (IgG4 + ) plasma cells and fibrosis. It can occur in almost all organs, commonly affecting the pancreas, biliary tract, salivary and lacrimal glands and kidneys. However, reports of IgG4-RD accompanied by pathologically confirmed, IgG4-related pleural disease are scarce. Here, we present a case of a 64-year-old man with suspected malignant pleural mesothelioma based on imaging findings but finally diagnosed with IgG4-RD (including pleuritis, periaortitis and bilateral submandibular gland enlargement) based on a high serum IgG4 level and pleural histopathological findings such as lymphoplasmacytic infiltration including IgG4 + plasma cells and fibrosis. Systemic corticosteroid therapy was effective at reducing serum IgG4, improving bilateral submandibular gland enlargement, and regressing pleural thickening and periaortic soft tissue. We also discuss clinical characteristics and pleural pathological features of previously reported cases with IgG4-related pleural disease based on a comprehensive literature review. Our case of IgG4-RD with pleura, aorta and submandibular gland involvement, pathologically confirmed by pleural specimen might be unique and very rare.

Aortitis is a rare entity that may cause fever of unknown origin. This entity has a wide various etiologies, which main cause is rheumatologic, but not only. Iatrogenia has also been described, including chemotherapy and supporting treatment (like granulocyte-colony stimulating factor in oncological patients. The evidence in favour of this pharmacological link is growing. The differential diagnosis of fever, in febrile neutropenia setting, can be difficult to itemize.

Cogan's syndrome (CS), a rare vasculitis characterized by non-syphilitic, interstitial keratitis and Ménière-like attacks, is classified into "typical" and "atypical" forms, while Takayasu arteritis (TAK) is a rare large-vessel vasculitis associated with human leukocyte antigen (HLA)-B*52. Very few cases meet both the CS and TAK classification criteria. We herein report a 53-year-old woman diagnosed with atypical CS and aortitis similar to TAK. Her 25-year-old daughter manifested TAK without symptoms of CS, and both are HLA-B*52 positive. Our case highlights the difficulties of distinguishing aortitis with atypical CS from aortitis with TAK.