UNASSIGNED: Chest pain is a frequent reason patients seek medical attention. The broad spectrum of potential etiologies makes determining the underlying cause of chest pain complex. Among cardiovascular etiologies, aortitis is a rare but life-threatening possibility that should be considered in the differential diagnosis.
UNASSIGNED: A 53-year-old female with a history of smoking presented with progressively worsening chest and epigastric pain over several weeks. She had seen multiple physicians previously for the same symptoms with unremarkable work-ups. Physical examination was notable for severe tenderness upon palpation of her lower abdomen. The electrocardiogram and troponins were unremarkable. Computed tomography of the abdomen revealed aneurysmal dilatation of the abdominal aorta, soft tissue thickening, and surrounding inflammatory stranding, consistent with aortitis. Infectious and autoimmune work-ups were unremarkable. Intravenous steroids were initiated, and her symptoms improved significantly. Her aortitis was attributed to inflammation secondary to chronic smoking.
UNASSIGNED: Aortitis is a rare condition with varied clinical presentations. Etiologies of aortitis include infection and non-infectious inflammation. Diagnosis of aortitis requires a thorough clinical assessment and prompt imaging of the aorta, with computed tomography being the preferred imaging modality.
UNASSIGNED: Evaluation for cardiovascular chest pain must extend beyond an electrocardiogram and troponin level. Imaging should be considered in patients with atypical symptoms. Aortitis is a rare but important diagnosis requiring immediate treatment.

Immune-checkpoint inhibitors (ICIs) are considered as the novel treatment modality in certain cancers. They may soon be used widely even as the first-line option for cancer treatment due to their remarkable efficacies and impacts on survival rates, particularly in cases of advanced metastatic cancer. Of note, these agents might unveil new autoimmune diseases as well as causing flare-ups of a pre-existing autoimmune disease. Data in this field have been accumulated during recent years. Early detection and a collaborative approach are, therefore, crucial in the management of a patient who presents with any of these conditions. Herein, we report a patient with a diagnosis of metastatic renal cell cancer presented with vasculitis involvement in the aorta during nivolumab treatment. Our aim with this case is to increase the awareness of ICI-related vasculitis involvement among rheumatologists in the light of literature.

OBJECTIVE: Systematic review of current evidence to analyze the prevalence of extracranial large vessel vasculitis (LVV) using 18F-FDG PET/CT in patients with polymyalgia rheumatica (PMR) or giant cell arteritis (GCA).
METHODS: PubMed and EMBASE were searched and the results were screened by two reviewers. Study quality was assessed using a modified version of the Newcastle-Ottawa scale. Heterogeneity between studies was assessed using the I2 statistic and the Q test. Further subgroup analyses were performed by disease type, study quality, and 18F-FDG PET/CT uptake criteria. Publication bias was assessed by funnel plot and Egger\'s test.
RESULTS: 268 publications were identified, of which 17 met the selection criteria and were included in the meta-analysis. The overall pooled prevalence of extracranial LVV by 18F-FDG PET/CT was 54.5% [95% CI: 42.6%-66.1%]. In patients with GCA the prevalence was significantly higher than in patients with PMR (60.1% vs. 41.8%, P = 0.006). Likewise, studies with a lower risk of bias reported a higher prevalence of extracranial LVV (61.1% vs. 46.9%; P = 0.010). No publication bias was observed.
CONCLUSIONS: The 18F-FDG PET/CT test may be useful in the detection of extracranial LVV, both in patients with PMR or GCA. Such involvement is more frequent in patients with GCA, and may vary depending on the quality of the studies.

An infectious aortic aneurysm is a rare disease entity. We report a challenging case of a 29-year-old male presenting with chest pain and constitutional symptoms. The patient was found to have three pseudoaneurysms of the aorta on imaging, significant pathological findings of necrotizing granulomatous lymphadenitis from a supraclavicular lymph node biopsy, and a highly suggestive clinical picture of tuberculous aortitis. He was referred to vascular surgery for intervention and discharged on antituberculous therapy for 6 months. To the best of our knowledge, only five cases of tuberculous aortic aneurysms have been reported from the Middle East and North Africa (MENA) region, all with favorable outcomes. A high index of suspicion, early detection, and prompt intervention are essential in managing such cases.

BACKGROUND: Endovascular repair of inflammatory abdominal aortic aneurysms (IAAAs) has emerged as an alternative to open surgery, but direct comparisons are limited. The aim of the study was to compare clinical outcomes of endovascular and open repair for IAAA according with specific clinical characteristics.
METHODS: We performed a literature review of reports describing patients who had open or endovascular repair for IAAA. A literature search was performed in June 2022 by 2 investigators who conducted a review of papers reported in PubMed, Embase, MEDLINE, and Cochrane Database. The strings \"Inflammatory aneurysm\" and \"Abdominal Aortic Aneurysms\" were used. There was no language restriction and screened reports were published from March 1972 to December 2021. We identified 2,062 patients who had open (1,586) or endovascular repair (476) for IAAA. Primary outcomes were operative mortality and morbidity. Secondary outcomes were complications during follow-up (mean follow-up: 48 months). Propensity score matching was performed between patients who had open or endovascular surgery.
RESULTS: In Western countries, propensity-weighted postoperative mortality (in-hospital) (1.5% endovascular vs. 6% open) and morbidity rates (6% vs. 18%) were significantly lower in patients who had endovascular repair (P < 0.0001); patients with larger aneurysm (more than 7 cm diameter), signs of active inflammation, and retroperitoneal rupture of the aneurysm had better outcomes after endovascular repair than after open surgery. Hydronephrosis was present in 20% of the patients. Hydronephrosis regressed in most patients when signs of active inflammation were present suggesting an acute onset of the hydronephrosis itself (fever, elevated serum C Reactive Protein) either after endovascular or open surgery. Long-standing hydronephrosis as suggested by the absence of signs of active inflammation rarely regressed after endovascular surgery despite associated steroid therapy. During a mean follow-up of 48 months, propensity-weighted graft-related complications were more common in patients who had endovascular repair (20% vs. 8%). For patients from Asia, short-term and medium-term results were similar after open and endovascular repair. IAAAs related with aortitis were more common in Asia. In Western countries, IAAAs were commonly associated with atherosclerosis.
CONCLUSIONS: Patients with IAAA represent a heterogeneous population, suggesting biological differences from continent to continent; conservative therapy and endovascular or open surgery should be chosen according to the patient clinical condition. Endovascular repair presents advantages in patients with signs of active inflammation and contained rupture of the IAAA and larger aneurysms. Hydronephrosis, without signs of active inflammation, rarely regresses after endovascular repair associated with steroid therapy. Further studies are needed to establish the long-term results of endovascular repair.

Description Vasculitides are a group of diseases that produces vasculitis, which is characterized by inflammatory infiltrates within blood vessel walls and results in intimal injury as well as progressive mural destruction. Infiltrates are characterized per the Chapel Hill classification, into large, medium, and small-vessel vasculitides. ANCA-associated vasculitis (AAV) is a disease that has been described as involving small-sized vessels. However, some cases of large vessel disease involvement have been documented. ANCA-associated aortitis is a rare entity poorly described in the literature. Due to the rarity of this pathology, there is no Level I evidence available regarding diagnosis and treatment. We present the rare case of an 80-year-old male presenting with ANCA-associated aortitis complicated by acute dissection of the left common iliac artery. His case was successfully managed by corticosteroid therapy and endovascular stenting of the involved iliac artery. ANCA-associated aortitis is a rare entity that has not been described well in the current literature. We believe this case to be the first involving ANCA-associated aortitis presenting with an acute dissection.

We herein report an 83-year-old woman with filgrastim-associated aortitis during chemotherapy for relapsed diffuse large B-cell lymphoma. She had been treated with filgrastim as a prophylaxis for neutropenia during the fourth cycle of chemotherapy from day 9 to 18. On day 21, she developed a fever. Contrast-enhanced computed tomography revealed aortitis of the descending aorta. The fever abated with non-steroidal anti-inflammatory drug treatment. A literature review identified a small number of aortitis cases all caused by prophylactic use of granulocyte colony-stimulating factors (G-CSFs), among which short-acting filgrastim was rarely encountered. The present and previous findings imply a possible relationship between aortitis and prophylactic G-CSF usage.

Although primarily a lung disease, extra-pulmonary tuberculosis (TB) can affect any organ or system. Of these, cardiovascular complications associated with disease or drug toxicity significantly worsen the prognosis. Approximately 60% of patients with TB have a cardiovascular disease, the most common associated pathological entities being pericarditis, myocarditis, and coronary artery disease. We searched the electronic databases PubMed, MEDLINE, and EMBASE for studies that evaluated the impact of TB on the cardiovascular system, from pathophysiological mechanisms to clinical and paraclinical diagnosis of cardiovascular involvement as well as the management of cardiotoxicity associated with antituberculosis medication. The occurrence of pericarditis in all its forms and the possibility of developing constrictive pericarditis, the association of concomitant myocarditis with severe systolic dysfunction and complication with acute heart failure phenomena, and the long-term development of aortic aneurysms with risk of complications, as well as drug-induced toxicity, pose complex additional problems in the management of patients with TB. In the era of multidisciplinarity and polymedication, evidence-based medicine provides various tools that facilitate an integrative management that allows early diagnosis and treatment of cardiac pathologies associated with TB.

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.

Inflammatory abdominal aortic aneurysms (InflAAAs) account for 5 - 10% of aortic aneurysms and are characterised by retroperitoneal fibrosis. Diagnosis is often delayed, and doubts remain about the optimal management strategy. This scoping review describes the current state of knowledge on InflAAAs.
Medline, PubMed, EMBASE, and Scopus were searched for relevant studies that evaluated the diagnosis and treatment of InflAAAs. The Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) protocol was followed.
Fifty-seven papers were selected (low level of evidence), which included 1 554 patients, who were mostly male and heavy smokers. A triad of chronic abdominal or back pain, weight loss, and elevated inflammatory markers was highly suggestive of the diagnosis but rarely present, and fever was noted only randomly. A mantle sign was seen on computed tomography angiography (CTA) in 73 - 100% of patients. Open surgical repair (OSR) and endovascular aortic aneurysm repair (EVAR) was reported in 1 376 and 178 patients, respectively. OSR was associated with significant iatrogenic bowel (n = 22), urinary tract system (n = 7), venous (n = 30), pancreatic (n = 6), and splenic (n = 5) injuries, while EVAR was associated with lower 30 day mortality (0 - 5% vs. 0 - 32%). One and two year mortality rates were similar between the two treatment modalities (0 - 20% and 0 - 36%, respectively). EVAR was more often associated with post-operative progression of inflammation (17% vs. 0.4%), and a higher frequency of persistent hydronephrosis (> 50%) and limb occlusion (20%). Used in < 10% of patients, corticosteroids led to complete pain relief and a reduction in peri-aortic inflammation within 6 - 18 months.
InflAAAs are characterised by non-specific symptoms, with the mantle sign on CTA being pathognomonic. Corticosteroids may be considered a basic treatment that all patients should receive initially. Low quality data indicate that EVAR (vs. OSR) is associated with fewer intra-operative complications and lower peri-operative mortality but more late fibrosis related adverse events. International multicentre registries are required to gather more insights into this challenging pathology.