Search Results (39)

Giant cell arteritis (GCA) is the most common primary systemic vasculitis of older adults and has traditionally been framed as an ophthalmologic emergency. Its most serious consequences, however, occur in the cardiovascular system, often years after inflammatory markers have normalized. In contemporary matched population cohorts, patients with GCA carry an approximately two-fold excess risk of aortic aneurysm; the historical Olmsted County cohort reported a 17-fold excess of thoracic aortic aneurysm with wide confidence limits, and the characteristic latency to aneurysm is five to ten years. Patients also face elevated risks of aortic dissection, myocardial infarction, stroke, venous thromboembolism, and heart failure that cluster within the first year of diagnosis, when vascular inflammation is most active. This narrative review, based on a structured search of PubMed/MEDLINE and Google Scholar through September 2026 with independent verification of every cited estimate, synthesizes current evidence on the full spectrum of cardiac and aortic complications of GCA, including aortitis, aneurysm and dissection, coronary arteritis, valvular disease, pericarditis, myocarditis, and conduction disease. We trace their common origin to the immunopathology of the arterial wall, where dendritic cell activation, Th1/Th17 polarization, and maladaptive vascular remodeling produce occlusion in medium-sized arteries and dilation in the aorta. We examine current diagnostic modalities, from the ultrasound-based fast-track clinic, which has reduced permanent visual loss by half or more in observational cohorts, to FDG-PET/CT, in which aortic uptake at diagnosis is associated with subsequent aortic dilation. We review treatment after the GiACTA and SELECT-GCA trials in the light of the 2025 European Alliance of Associations for Rheumatology (EULAR) recommendations, the unresolved role of antiplatelet and statin therapy, and the surgical and endovascular management of GCA-related aortopathy. We suggest that the organization of care, as well as the limitations of drug therapy, may contribute to the residual mortality of GCA, a hypothesis that has not been tested; distinguish recommendations that are supported by current guidelines from those that are our own proposals; and outline a cardio-rheumatology pathway in which baseline vascular phenotyping informs individualized aortic surveillance. GCA is a disease whose natural history often begins in the rheumatology clinic and ends in the cardiac operating room without prior surveillance. Full article

Aortopathies in autoimmune rheumatic diseases (ARD) include a spectrum of aortic pathologies—including aortitis, aneurysms, dissections, and insufficiency—primarily caused by systemic inflammation. This comprehensive review investigates the clinical manifestations, pathophysiology, diagnostic modalities, and management strategies across various rheumatic diseases associated with aortopathies such as large vessel vasculitis (e.g., Takayasu arteritis, giant cell arteritis), connective tissue diseases (e.g., systemic lupus erythematosus, rheumatoid arthritis, ankylosing spondylitis, systemic sclerosis) and less common conditions (e.g., relapsing polychondritis, Cogan’s syndrome, Behçet’s disease, IgG4-related disease). Disease-specific pathophysiologic mechanisms of aortic wall inflammation and remodeling, including granulomatous and lymphoplasmacytic patterns and mixed inflammatory infiltrates, are described. Diagnostic imaging modalities—such as CTA, MRI, and PET/CT—are evaluated for their roles in detecting active inflammation, assessing structural complications, and guiding clinical decision-making. Histopathological findings provide insight into disease-specific vascular changes. Management strategies focus on the use of glucocorticoids, disease-modifying antirheumatic drugs (DMARDs), and biologics, including IL-6 and TNF-α inhibitors, with an emphasis on patient-centered approaches, multidisciplinary care, and timely surgical intervention for complications. Evidence gaps include optimal screening intervals and the role of novel biomarkers in risk stratification and in monitoring disease progression, highlighting the need for early recognition, frequent monitoring, and aggressive management of aortic involvement in rheumatic diseases to prevent life-threatening complications. Full article

Aortitis—inflammation of the aortic wall—presents at the interface of vasculitis, infection, structural aortic disease, and cardiovascular risk. It may occur in giant cell arteritis (GCA), Takayasu arteritis, immunoglobulin G4 (IgG4)-related disease, drug-induced injury, infection, or as an isolated finding after aortic surgery. Modern imaging detects aortic inflammation more frequently, but the main challenge is classification rather than detection: determining whether disease is infectious or immune-mediated, active or dominated by fixed structural damage, systemic or isolated, and whether the dominant threat is aneurysm, dissection, undertreated infection, or avoidable immunosuppression. This review considers aortitis as a high-risk vascular syndrome requiring aetiology-first classification rather than descriptive labelling. Before escalating immunosuppression, infection must be actively excluded and inflammatory activity distinguished from fixed vascular damage. Treatment should be individualised according to phenotype, age, vascular territory, comorbidity, and toxicity risk, with surveillance continuing even after symptoms and inflammatory markers improve. Optimal care depends on multidisciplinary assessment integrating rheumatology, infectious diseases, vascular surgery, radiology, and cardiology expertise. Progress will require standardised imaging definitions, registries linking inflammatory control with structural vascular outcomes, and validation of artificial intelligence (AI) tools before clinical adoption. Full article

Erdheim–Chester Disease (ECD) constitutes a rare and clinically heterogeneous non-Langerhans cell histiocytosis, characterized by the systemic infiltration of tissues by foamy, lipid-laden histiocytes. These cells typically exhibit an immunophenotypic profile positive for CD68 and negative for CD1a. The disease’s multifaceted presentation, which can span from isolated bone lesions to fulminant multi-organ failure, frequently results in considerable diagnostic delay. In this case-based review, we describe the case of a 58-year-old who presented with a primary complaint of exertional dyspnoea and fatigue. The initial diagnostic evaluation revealed a hemodynamically significant circumferential pericardial effusion and imaging findings suggestive of aortitis. Clinical presentation of ECD depends on the organs and tissues involved, and may range from bone pain to neurological symptoms, endocrine dysfunction, and cardiac involvement. Cardiovascular involvement occurs in at least 40% of ECD patients, although it is frequently underdiagnosed. Cardiac ECD is heterogeneous and may mimic many alternative aetiologies. The infiltration of the right atrioventricular sulcus, right atrial walls, or interatrial septum is one of the most typical cardiac manifestations of ECD. Recognition of pseudo-tumour intra-atrial mass, pericardial involvement, as well as the circumferential encasement of the entire aorta, the so-called coated aorta, are other frequent findings. Diagnosis often requires a multimodal approach, in particular when cardiac symptoms represent the onset of clinical manifestation of ECD. The combined use of computed tomography, fluorodeoxyglucose positron emission tomography, dedicated cardiac and abdominal magnetic resonance imaging, and X-ray of long bones can collectively reveal a constellation of findings diagnostic of ECD. Full article

Arterial wall fragility represents a unifying pathophysiological substrate underlying a broad spectrum of aortic diseases, including aneurysms, dissections, intramural hematoma, penetrating atherosclerotic ulcers, and aortitis. Rather than distinct entities, these conditions increasingly appear as interconnected manifestations of impaired wall integrity and maladaptive vascular remodeling. This narrative review provides a structured overview of the imaging correlates of arterial wall fragility from a CT-centered, multimodality perspective. Computed Tomography Angiography (CTA) remains the first-line imaging modality in acute settings, enabling rapid and comprehensive assessment of vascular anatomy, luminal integrity, and the presence of life-threatening complications. Complementary modalities, including magnetic resonance imaging and ultrasound, contribute additional information on tissue characterization and hemodynamic evaluation in selected stable patients, follow-up settings, or specific clinical scenarios. Across imaging modalities, specific features—such as false lumen patency, intramural hematoma characteristics, ulcer-like projections, aneurysm morphology, and periaortic inflammatory changes—have been reported as markers of wall instability. These imaging-derived findings may provide clinically relevant information beyond traditional diameter-based assessment and support more refined risk stratification. Emerging approaches, including artificial intelligence, radiomics, computational modeling, and advanced MRI techniques, are expanding the role of imaging toward quantitative evaluation. However, their routine clinical implementation still requires standardization and prospective validation. Overall, a CT-centered multimodality imaging strategy may support a more comprehensive assessment of arterial wall fragility and contribute to individualized clinical decision-making in patients with aortic disease. Full article

Background/Objectives: Lyme disease is a tick-borne zoonosis caused by Borrelia burgdorferi that can affect multiple organ systems. Although cardiovascular involvement is considered uncommon, it may lead to severe and potentially life-threatening complications, particularly conduction disturbances and inflammatory cardiac conditions. This review aims to describe the spectrum of cardiovascular manifestations documented in patients with Lyme disease, focusing on clinical presentation, diagnostic approaches, management strategies, and reported outcomes. Methods: A narrative literature review was performed using PubMed, MEDLINE, and Google Scholar. Articles published between January 2000 and July 2025 in English or Spanish were screened. Eligible studies included original research articles, systematic and narrative reviews, case series, and case reports describing confirmed Lyme disease with cardiovascular involvement. A total of 30 studies were included. The available evidence was predominantly based on case reports and small case series, with considerable heterogeneity in study design, patient populations, and reported outcomes. Data on clinical manifestations, diagnostic methods, treatment strategies, and outcomes were extracted and synthesized. Results: Atrioventricular conduction disturbances were the most frequently reported cardiovascular manifestation, ranging from first-degree block to complete heart block, often presenting abruptly with syncope or bradycardia. Other reported manifestations included atrial and ventricular arrhythmias, myocarditis, pericarditis, myopericarditis, valvular endocarditis, aortitis, and vasculitis. Diagnosis relied on a combination of clinical suspicion, epidemiologic exposure, serologic testing, electrocardiographic monitoring, and cardiac imaging. Most patients were treated with antimicrobial therapy, commonly intravenous ceftriaxone followed by oral doxycycline, with temporary pacemaker support required in selected cases. Overall, clinical outcomes were favorable when treatment was initiated promptly. Conclusions: Cardiovascular involvement in Lyme disease, although infrequent, encompasses a broad clinical spectrum with potentially serious consequences. Early recognition, appropriate diagnostic evaluation, and timely antimicrobial therapy are essential to ensure reversibility of cardiac manifestations and favorable outcomes. However, the available evidence is limited by heterogeneity and the predominance of low-level-evidence studies. Full article

Background: Plasma cell granuloma is generally considered a pseudotumor formed by reactive, polyclonal plasma cells. Although most cases can show polyclonal gammaglobulin production, quite a minority may exhibit monoclonal gammopathy, which mimics plasma cell neoplasms such as multiple myeloma or plasmacytoma. Because of this overlap, distinguishing reactive monoclonal proliferation from true malignancy is clinically essential. Case report: A 79-year-old man was presented with an anterior chest wall mass that had grown during investigation for fever of unknown origin. ¹⁸F-fluorodeoxyglucose positron emission tomography (FDG-PET) revealed a sternal bone mass (SUVmax 9.04), aortic uptake of bifurcation (SUVmax 7.08), and Th7/8 soft tissue mass (SUVmax 5.32). Results from the FDG-PET revealed infectious reactions. A chest wall biopsy revealed high degree proliferation of plasma cells. Hematologists suspected plasmacytoma. The pathologist did not diagnose plasmacytoma; thus, there remains a possibility of reactive granuloma lesion. Lastly, the patient’s vertebral soft tissue mass culture yielded Staphylococcus aureus. The patient was treated with antimicrobials and responded well. Discussion: In the presented case, FDG-PET revealed an aortic mass with an aortic aneurysm, a sternal mass, and a vertebral mass, as multiple lesions. The abscess lesions that initially resembled multiple plasmacytomas were identified as plasma cell granuloma. The final diagnosis required demonstrating biopsy and definitive monoclonality. Light-chain restriction or monoclonal protein should be considered in the clinical context. Ultimately, this case highlights the diagnostic value of FDG-PET and the importance of differentiating reactive plasma cell granuloma from true plasma cell neoplasm to guide appropriate management. In conclusion, a reactive plasma cell granuloma associated with infectious aortitis can exhibit monoclonal gammopathy, mimicking plasma cell neoplasm. Careful pathological and clinical evaluation is essential to avoid misdiagnosis and ensure proper treatment. Full article

Background/Objectives: This study examined the clinical utility of diffusion-weighted whole-body magnetic resonance imaging with background body signal suppression (DWIBS) for differentiating infectious from non-infectious aortitis. Methods: The study included 32 patients with suspected inflammatory aortitis who underwent non-contrast computed tomography (NCCT) and magnetic resonance imaging. We evaluated the diagnostic performance of DWIBS using the spinal cord as a reference, NCCT, and their combination. The diagnosis of infectious aortitis was adjudicated based on imaging, clinical, and laboratory findings. We conducted a sensitivity analysis using a stricter definition of infectious aortitis that required both surgical and microbiological confirmation. Results: Fifteen patients were diagnosed with infectious aortitis. The sensitivity, specificity, and areas under the receiver operating characteristic curves were 93.3%, 70.6%, and 0.82, respectively, for NCCT; 93.3%, 76.5%, and 0.85, respectively, for DWIBS; and 86.7%, 94.1%, and 0.90, respectively, for the combination of both modalities. In the sensitivity analysis, the combined DWIBS and NCCT approach demonstrated a specificity of 87.5% and a sensitivity of 70.8%. Conclusions: DWIBS using the spinal cord as a reference appears to be a promising diagnostic tool for differentiating infectious from non-infectious aortitis, especially when combined with NCCT. Full article

Background and Clinical Significance: Fever of undetermined origin (FUO is a diagnostic challenge. It is essential to exclude infections, paraneoplastic syndromes, and large-vessel vasculitis (LVV). Case presentation: We describe a 59-year-old female with FUO and no apparent signs of infection. Laboratory results were consistent with inflammation (ESR 83, CRP 203 (ref. value (RV) < 5 mg/dl), ferritin 311 (RV < 120 µg/L), microcytic anemia, thrombocytosis. With administration of both ceftriaxone and levofloxacin, a decrease in CRP was monitored (51 mg/L). HRCT of the chest, abdomen, and pelvis revealed a saccular aneurysm of the descending thoracic aorta and an ectatic right common iliac artery. Due to suspicion of LVV, CT angiography was performed to exclude inflammatory changes in the blood vessels. Diffuse atherosclerosis with a 30 mm penetrating thoracic aortic ulcer (PAU) was found. PET-CT and leukocyte scintigraphy were used to rule out vasculitis and infection. The patient was successfully treated with Thoracic Endovascular Aortic Repair (TEVAR). Conclusions: As sophisticated imaging techniques become more widely used, more PAUs are being detected as incidental abnormalities in individuals without acute aortic syndrome. With adequate management, many people with PAU can live a stable and healthy life without experiencing significant consequences. Full article

Background: Data on the incidence of IgG4-related disease (IgG4-RD) are scarce. Our aim was to determine the incidence of IgG4-RD in a well-defined region. Methods: This retrospective study covered the Ljubljana region over the period from January 2012 to December 2024. A review of cases diagnosed with IgG4-RD was performed at several departments of the University Medical Centre Ljubljana—an integrated secondary/tertiary university teaching hospital (rheumatology, nephrology, angiology, gastroenterology, abdominal surgery, ENT surgery, ophthalmology). While IgG4-RD cases at the Department of Rheumatology were collected prospectively, potential cases at other departments were retrieved by searching electronic medical database for the keyword “IgG4”. In addition, the Institute of Pathology, Faculty of Medicine, University of Ljubljana, provided a list of patients with histological features consistent with IgG4-RD. Year-specific incidence rates and an average incidence rate over the 13-year period were determined. Clinical features of patients were analysed. Results: During the observation period, 58 cases of IgG4-RD were diagnosed. Of these, 35 patients were residents of the Ljubljana region, which had an average adult population of 541,600. The estimated average annual incidence rate of IgG4-RD was 5.0 per million (95% confidence interval: 3.5; 6.9), with year-specific incidence rates fluctuating between 1.8 and 9.3 per million adults. The cases were stratified into four phenotypic categories: pancreato-hepato-biliary (17%), retroperitoneal fibrosis-aortitis (43%), head and neck-limited (14%), and Mikulicz syndrome with systemic involvement (26%). Conclusions: The average annual incidence rate of IgG4-RD was 5 per million adults, with the retroperitoneal fibrosis-aortitis phenotype predominating in our cohort. Full article

A 75-year-old man, with a history of descending thoracic aortic rupture and dissection treated with aortic stenting at 73 years old, was admitted for rehabilitation following recurrent cerebral ischemic attacks. Upon admission, blood tests revealed elevated inflammatory markers, including a C-reactive protein (CRP) level of 10.75 mg/dL and a D-dimer level of 4.2 µg/mL, alongside microcytic anemia. Despite thorough evaluations using computed tomography (CT) and ultrasound, the origin of these abnormalities remained unidentified. Two months later, MRI using diffusion-weighted whole-body imaging with background body signal suppression (DWIBS) revealed hyperintensities in the thoracic aorta. He remained asymptomatic and progressed well during rehabilitation, prompting continued observation. However, three months after admission, he developed hemoptysis. Contrast-enhanced CT showed pneumonia, as well as enhanced lesions in the aortic wall, confirming aortic inflammation. Due to concerns about aortic stent ulceration, an emergency stent graft insertion extending to the superior mesenteric artery was performed. He recovered uneventfully and was discharged. DWIBS is an MRI-based tool that avoids exposure to radiation or contrast agents and is cost-effective. MRI using DWIBS demonstrated high signal accumulations in the aortic wall, indicative of inflammation. These findings suggest that DWIBS holds significant potential as a powerful imaging tool for detecting and assessing inflammation, particularly in the aorta. Full article

Aortitis, defined as inflammation of the aorta, can lead to aneurysms and dissections. Intra-operative sampling is essential for diagnosis, with many cases presenting asymptomatically as clinically isolated aortitis. Previous studies investigating aortitis in major aortic surgery have been limited by low intra-operative sampling. We performed an 11-year, retrospective, cross-sectional study to investigate the true prevalence of aortitis in thoracic aortic aneurysms and dissections by analysing all major aortic operations performed in a single centre. We collected medical histories, histological reports, post-operative outcomes and follow-up data; 537 patients met the inclusion criteria, representing an 88% histological sampling rate. The prevalence of aortitis was 10.6% (n = 57), of which 75% were clinically isolated. The re-operation rate in aortitis was twice that of non-aortitis patients (17.5% vs. 9.4%, p = 0.054). Multivariate logistic regression identified increased age, female sex, current smoking, and other inflammatory diseases as significantly associated with aortitis, with a bicuspid aortic valve associated with a significantly decreased likelihood of aortitis. The true prevalence of aortitis is likely higher than reported in previous studies, with our study showing twice the prevalence found in previous studies with lower sampling rates. Due to the increased re-intervention in aortitis, specialist multi-disciplinary follow-up and aortitis centres should be formed. Full article

Background: Antineutrophil cytoplasm antibody (ANCA)-associated vasculitis usually affects small blood vessels and is characterized by the presence of circulating autoantibodies (c-ANCA or p-ANCA). The risk of cardiovascular events is threefold higher compared to general population, and cardiac manifestations include myocarditis, pericarditis, valvulitis, aortitis, or coronary arteritis. Coronary involvement is very rare, but it is a potentially life-threatening manifestation. Methods: We present an atypical cardiac scenario of p-ANCA vasculitis. Results: A 68-year-old woman with known p-ANCA vasculitis and stage 5 chronic kidney disease (CKD) on hemodialysis presented with dizziness accompanied by low blood pressure and chest pain. Electrocardiogram on arrival showed slightly ST-T changes, with negative cardiac biomarkers and no abnormalities in cardiac regional wall motion. Five hours after presentation, the patient repeated chest pain, accompanied by a drop in blood pressure and junctional escape rhythm. The highly sensitive cardiac troponin I (hs-cTnI) was raised at 560 ng/L. Coronary angiography showed coronary arteries without significant stenosis. The provocative test with intracoronary ergonovine demonstrated coronary vasospasm of the anterior descending artery accompanied by chest pain, with resolution after intracoronary nitroglycerin. Under amlodipine, nitrate, acetylsalicylic acid, statin and corticosteroids the patient did not experience the recurrence of angina. Conclusions: This case illustrates coronary involvement, manifested as coronary spasm with favorable outcomes, in systemic vasculitis. The underlying mechanism is immune-mediated inflammation in vascular walls. Full article

Introduction: We conducted a comprehensive comparative analysis of the Okazaki, Umehara, and American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) classification criteria for diagnosing immunoglobulin G4-related disease (IgG4-RD). Materials and Methods: A retrospective study was conducted in a single tertiary hospital, using expert clinical judgment as the gold standard. We compared the diagnostic accuracy of the Okazaki, Umehara, and ACR/EULAR criteria in a cohort of 41 patients with suspected IgG4-RD. We assessed sensitivity, specificity, and positive and negative predictive values for each criterion, and conducted a separate analysis based on four IgG4-RD subtypes. Results: A total of 30 patients were confirmed to have IgG4-RD and 11 were identified as mimickers. The Umehara criteria demonstrated the highest sensitivity (83.33%), followed by the ACR/EULAR 2019 (66.67%) and Okazaki (60.0%) criteria. All three criteria exhibited 100% specificity, with overall diagnostic accuracy ranging from 70% to 88%. The areas under the curve (AUC) were 0.917 (Umehara), 0.800 (Okazaki), and 0.833 (ACR/EULAR 2019), indicating significant diagnostic effectiveness (p < 0.000). Subtype analysis revealed that the Umehara and ACR/EULAR 2019 criteria were more effective in diagnosing pancreato-hepato-biliary involvement (subtype 1), while the Okazaki and ACR/EULAR 2019 criteria were more effective in diagnosing retroperitoneal fibrosis and/or aortitis (subtype 2). Conclusions: Our study provides valuable insights into the diagnostic performance of the Okazaki, Umehara, and ACR/EULAR criteria for a cohort of patients with suspected IgG4-RD. The Umehara criterion demonstrated the highest sensitivity, suggesting its potential utility for screening purposes, while all three criteria showed consistent specificity. Full article

Background/Objectives: Imaging studies have transformed the diagnosis of large vessel vasculitis (LVV) involvement in giant cell arteritis (GCA). A positron emission tomography/computed tomography (PET/CT) scan with 18-fluorodeoxyglucose (18F-FDG) has emerged as a valuable tool for assessing LVV. We aimed to determine the utility of an 18F-FDG-PET/CT scan in detecting LVV in GCA in the ARTESER registry. Methods: The ARTESER study is a large multicenter, retrospective, longitudinal, and observational study, promoted by the Spanish Society of Rheumatology. It included patients newly diagnosed with GCA across 26 tertiary hospitals from 1 June 2013 to 29 March 2019. Patients with a diagnosis of incidental GCA were included if they fulfilled specific criteria, including the ACR 1990 criteria, positive imaging examinations, or the expert clinical opinion of investigators. Differences between patients with positive and negative 18F-FDG-PET/CT scan results were analyzed using a bivariate model. A regression model assessed associations in patients with a positive scan, and the predictive capacity of the cumulative dose of glucocorticoids (GC) on PET scan outcomes was evaluated using ROC curve analysis. Results: Out of 1675 GCA patients included in the registry, 377 met the inclusion criteria of having an 18F-FDG-PET/CT scan. The majority were diagnosed with a cranial GCA phenotype, and 65% had LVV. The thoracic aorta was the most frequently affected. Cardiovascular disease, diabetes, and older age had a negative association with a positive scan outcome. The OR for having a positive 18F-FDG-PET/CTC scan was lower as the number of days increased. Depending on the cumulative dosage of the GC, the 18F-FDG-PET/CT scan showed an AUC of 0.74, with a Youden index > 60 mg/day. Conclusions: Younger patients showed a higher probability of presenting LVV as detected by the 18F-FDG-PET/CT scan. The timing of the examination and the cumulative dosage of the GC influenced the likelihood of a positive result, with earlier tests being more likely to detect inflammation. Full article