vasculitis – ABC CARDIOL

ABC

Editorial

Beyond Remission: The Cardiometabolic Burden of ANCA-Associated Vasculitis

The intersection between systemic inflammation and cardiometabolic disease has emerged as a defining paradigm in contemporary medicine. Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV)—including granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA)—represent a particularly compelling model where immune-mediated vascular injury coexists with a substantial and often underrecognized metabolic burden. In this issue, Bezerra et al. provide important data regarding the prevalence and predictors of metabolic syndrome (MetS) within this population.

Using the NCEP ATP III criteria, the authors report a MetS prevalence of 43.5% in a cohort of 62 consecutive AAV patients followed at a Brazilian tertiary referral center, compared with only 13.2% in age- and sex-matched controls (p=0.001). In multivariable analysis restricted to AAV patients, male sex (OR=11.43; 95%CI=2.81–46.52) and higher body mass index (OR=1.13; 95%CI=1.02–1.25) emerged as independent predictors of MetS. These findings align with prior reports in AAV populations, where MetS prevalence has ranged from 43% to 51%,^– mirroring patterns seen across a broad spectrum of systemic autoimmune rheumatic diseases, including systemic lupus erythematosus, rheumatoid arthritis, and systemic sclerosis.^,

[…]

Read Full Article

ABCIC

Case Report

Behcet’s Disease with Vascular Involvement: Case Report

Isabela Rodrigues Tavares et al. Maria Cristina Miranda Ana Claudia Gomes Pereira Petisco Daniela Souto Barros … Caio Buscatti Folino Vitor Leonidas Boher Dornas Mohamed Hassan Saleh Fábio Henrique Rossi Raquel Peres Sousa Larissa Chaves Nunes Carvalho

Clinical case

Male patient, 34 years of age, previously healthy, admitted to the Emergency Room (ER) of Instituto Dante Pazzanese de Cardiologia reporting a 3-month history of pain in the right lower limb (RLL) and limping for distances of 100 meters in the 3 weeks prior to admission. The patient evolved with worsening pain in the right calf and spontaneous pulsatile bulging a week before admission. He mentioned the use of Rivaroxaban for deep venous thrombosis (DVT) in the RLL five months before, weight loss (15kg in 6 months), and appearance of aphthous lesions in the oropharyngeal region. He had a vascular ultrasound image from another service, with signs of DVT in a partially recanalized right popliteal vein.

On the occasion of admission, physical examination indicated: posteromedial pulsatile mass on the right leg and present pulses, except posterior tibial. Heart, lungs, abdomen, and left lower limb (LLL) without alterations.

[…]

Read Full Article

ABCIC

Case Report

The Value of Vascular Ultrasonography in Defining Inflammatory Activity in Takayasu Arteritis: Case Reports

Fanilda Souto Barros et al. Simone Nascimento dos Santos Joana Storino Cláudia Maria Vilas Freire … Felipe Souto Barros Valquiria Garcia Dinis

Introduction

Takayasu arteritis (TA) is a rare large-vessel arteritis that primarily affects the aorta and its major branches. The greatest challenge is to identify disease activity, since therapeutic measures modify the clinical course of the disease.

Vascular ultrasonography (VUS) is a promising tool for characterizing vessel wall inflammation and monitoring hemodynamic changes in response to therapy.

[…]

Read Full Article

ABC

Original Article

Exercise Testing In Patients with Sickle Cell Disease: Safety, Feasibility and Potential Prognostic Implication

Christiano Gonçalves de Araújo et al. Maria Betânia Solis Resende Julia Teixeira Tupinambás Rebeca Coeli Teodoro Maciel Dias … Flávio Coelho Barros Maria Carmen Melo Vasconcelos José Nelio Januário Antonio Luiz Pinho Ribeiro Maria Carmo P. Nunes

Abstract

Background

Patients with sickle cell disease (SCD) are at increased risk for cardiovascular complications. Exercise testing is used as a prognostic marker in a variety of cardiovascular diseases. However, there is a lack of evidence on exercise in SCD patients, particularly regarding its safety, feasibility, and possible prognostic role.

Objectives

We used the maximal treadmill test to determine safety and feasibility of the exercise testing in SCD patients. Additionally, the factors associated with exercise duration, as well as the impact of exercise-induced changes on clinical outcome, were also assessed.

Methods

One-hundred thirteen patients with SCD, who underwent exercise testing, were prospectively enrolled. A comprehensive cardiovascular evaluation, including echocardiography and B-type natriuretic peptide (BNP) levels, were obtained. The long-term outcome was a composite endpoint of death, severe acute painful episodes, acute chest syndrome, or hospitalization for other SCD-related complications. Cox regression analysis was performed to identify the variables associated with the outcome. A p-value<0.05 was considered to be statistically significant.

Results

The mean age was 36 ± 12 years (range, 18-65 years), and 62 patients were women (52%). Ischemic electrocardiogram and abnormal blood pressure (BP) response to exercise were detected in 17% and 9%, respectively. Two patients experienced pain crises within 48 hours that required hospitalization. Factors associated with exercise duration were age, sex, tricuspid regurgitation (TR) maximal velocity, and E/e’ ratio, after adjustment for markers of disease severity. During the mean follow-up of 10.1 months (ranging from 1.2 to 26), the endpoint was reached in 27 patients (23%). Independent predictors of adverse events were hemoglobin concentration, late transmitral flow velocity (A wave), and BP response to exercise.

Conclusions

Exercise testing in SCD patients who were clinically stable is feasible. Exercise duration was associated with diastolic function and pulmonary artery pressure. Abnormal BP response was an independent predictor of adverse events.

Read Full Article

IJCS

Case Report

Surgical Repair of a Ruptured Giant Abdominal Aortic Aneurysm in a 16-Year-Old with Takayasu’s Arteritis: Case Report and Etiological Review

José Maciel Caldas dos Reis et al. Glauco dos Santos Melo Murilo Vasconcelos de Oliveira Felipe Eduardo de Oliveira Santos … Tereza Maria Meireles Fernandes da Silva Hugo Luis da Silva Ferreira

Abstract

Takayasu’s arteritis is a type of primary systemic vasculitis that affects medium and large arteries, including the aorta and its main branches, as well as the pulmonary and coronary arteries. Although rare in children, it is the third most common vasculitis in the pediatric population, often with delayed diagnosis due to the nonspecific presentation of clinical symptoms in its initial phase. This is a case of a 16-year-old girl with a giant ruptured abdominal aortic aneurysm, who needed surgery on an emergency basis. The etiological aspects involved in aneurysms in young patients are also addressed.

Read Full Article

ABC

Editorial

Kawasaki Disease: Predictors of Intravenous Immunoglobulin Resistance and Cardiac Complications: New Perspectives?

Isabel Cristina Britto Guimarães

Kawasaki disease (KD) is an acute inflammatory disease of unknown origin and associated with vasculitis. It affects medium-sized vessels. Coronary artery abnormalities (CAA) — aneurysms or dilations — are the main complications of KD, and are currently the most common cause of heart disease acquired in children in developed countries. In the acute phase, the initial standard treatment recommended is intravenous immunoglobulin (IVIG) and aspirin, to reduce the risk of damage to the coronary arteries. However, 10 to 20% of children with KD do not respond to the initial standard treatment, with recurrent fever within 36 to 48 hours after IVIG infusion. Studies show that the likelihood of KD patients resistant to IVIG to have coronary artery lesions is nine times greater than those sensitive to IVIG. This suggests that there is a critical window to block the inflammatory process and prevent CAA in the long term.

The study by Faim et al. aimed to identify predictive factors for resistance to intravenous immunoglobulin (IVIG), calculate the effectiveness of Japanese predictive models and characterize the cardiac complications of patients followed up at a single institution.

[…]

Read Full Article

ABC

Image

Takayasu Arteritis: From Diagnosis to a Life-Threatening Complication

Filipa Cordeiro et al. Sofia Silva Carvalho Fernando Salvador Alberto Ferreira J. Ilidio Moreira

A fifty-two-year-old Caucasian woman was admitted for severe epigastric pain irradiating to the back. Physical examination and electrocardiogram were normal. Laboratory tests showed leucocytosis (11100 cells/uL) and increased levels of C-reactive protein (15.6 mg/dl). Due to the suspicion of acute aortic syndrome (AAS), she underwent computed tomography (CT), which showed a low attenuation circumferential mural thickening of the aorta (43 Hounsfield units (HU)), which enhanced (73 HU) after contrast administration (-), suggestive of aortitis. Transesophageal echocardiogram also revealed thickened thoracic aorta (). Cardiovascular magnetic resonance imaging confirmed the diagnosis of aortitis and excluded intramural hematoma (mural thickening hypointense on T1-weighted images and hyperintense on T2-weighted images)^, (Figures E-F). Infectious serologies were negative.

[…]

Read Full Article

ABC

Artigo Original

Vasculitides and eosinophils in emdomyocardial biopsies as rejection predictors in heart transplantation

Reginaldo Cipullo et al. Marco Aurélio Finger João Manoel Rossi Neto Carlos Mendes Contreras … Nádia Van Geen Poltronieri Mabel de Moura Barros Zamorano Lílian Mary da Silva Paulo Chaccur Jarbas Jackson Dinkhuysen Noedir Antônio Groppo Stolf

BACKGROUND: The clinical significance of vasculitides, ischemic lesions, Quilty effect and the presence of eosinophils in endomyocardial biopsies of heart transplantation recipients with mild rejection has yet to be established. OBJECTIVE: To verify whether these histological findings observed in endomyocardial biopsies (eosinophils, vasculitides, Quilty effect and ischemic lesions) are capable of predicting acute graft rejection. METHODS: A total of 1,012 consecutive endomyocardial biopsies were reevaluated; of these, 939 were classified as OR or 1R according to the Nomenclature of the International Society of Heart and Lung Transplantation of 2005 and divided in two groups: (1) Predictive biopsies: those that preceded acute rejection; and (2) Nonpredictive biopsies: those that did not precede acute rejection. We compared the occurrence of the following histological findings: vasculitides, ischemic lesions, Quilty effect and eosinophils between the groups by uni- and multivariate analyses. RESULTS: The statistical analysis showed that the presence of severe vasculitides and eosinophils were the best predictors for future acute rejection, with the following odds ratios: 10.60 (95%CI: 3.62 – 31.06. p < 0.001) and 6.26 (95%CI: 3.16 – 12.43, p < 0.001). CONCLUSION: Severe vasculitides and eosinophils in myocardial biopsies are the main predictive factors of acute graft rejection post-heart transplantation.

Read Full Article

ABC

Case Report

Takayasu's disease with severe heart and arterial involvement in a preschool-age child

Fátima Derlene da Rocha Araújo et al. Zilda Maria Alves Meira Fabiana Arantes de Araújo Glaucia Maria Vasconcelos Severiano Kelly Nascimento Brandão

The authors describe a case of Takayasu’s arteritis in a child of only 3 years of age, emphasizing the rarity of this disease in this age group. The child was admitted to the emergency room in a post-convulsive state after a tonic-clonic seizure. After a detailed clinical examination, extensive diagnostic tests, and observation of the clinical evolution, the diagnosis was Takayasu’s disease with severe cardiac and arterial involvement. The report warns pediatricians and cardiologists to awareness of the possibility of this disease in very small infants, in countries where it is underdiagnosed.

Read Full Article