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The Journal of Rheumatology Oct 2021To assess the spectrum and long-term outcome of patients with noninfectious aortitis.
OBJECTIVE
To assess the spectrum and long-term outcome of patients with noninfectious aortitis.
METHODS
We performed a retrospective multicenter study of 353 patients (median age at diagnosis was 62 [IQR 46-71] yrs and 242 [68.6%] patients were women) with noninfectious aortitis. Factors associated with vascular complications were assessed in multivariate analysis.
RESULTS
We included 136 patients with giant cell arteritis (GCA), 96 with Takayasu arteritis (TA), 73 with clinically isolated aortitis (CIA), and 48 with aortitis secondary to inflammatory diseases (including Behçet disease, relapsing polychondritis, IgG4-related disease, Cogan syndrome, ankylosing spondylitis). After a median follow-up of 52 months, vascular complications were observed in 32.3%, revascularizations in 30% of patients, and death in 7.6%. The 5-year cumulative incidence of vascular complications was 58% (95% CI 41-71), 20% (95% CI 13-29), and 19% (95% CI 11-28) in CIA, GCA, and TA, respectively. In multivariate analysis, male sex (HR 2.10, 95% CI 1.45-3.05, < 0.0001) and CIA (HR 1.76, 95% CI 1.11-2.81, = 0.02) were independently associated with vascular complications.
CONCLUSION
Noninfectious aortitis accounts for significant morbidity and mortality. CIA seems to carry the highest rate of vascular complications.
Topics: Aortitis; Female; Giant Cell Arteritis; Humans; Male; Polychondritis, Relapsing; Retrospective Studies; Takayasu Arteritis
PubMed: 34210830
DOI: 10.3899/jrheum.201274 -
Frontiers in Immunology 2020Autoimmune and autoinflammatory diseases of the medium and large arteries, including the aorta, cause life-threatening complications due to vessel wall destruction but... (Review)
Review
Autoimmune and autoinflammatory diseases of the medium and large arteries, including the aorta, cause life-threatening complications due to vessel wall destruction but also by wall remodeling, such as the formation of wall-penetrating microvessels and lumen-stenosing neointima. The two most frequent large vessel vasculitides, giant cell arteritis (GCA) and Takayasu arteritis (TAK), are HLA-associated diseases, strongly suggestive for a critical role of T cells and antigen recognition in disease pathogenesis. Recent studies have revealed a growing spectrum of effector functions through which T cells participate in the immunopathology of GCA and TAK; causing the disease-specific patterning of pathology and clinical outcome. Core pathogenic features of disease-relevant T cells rely on the interaction with endothelial cells, dendritic cells and macrophages and lead to vessel wall invasion, formation of tissue-damaging granulomatous infiltrates and induction of the name-giving multinucleated giant cells. Besides antigen, pathogenic T cells encounter danger signals in their immediate microenvironment that they translate into disease-relevant effector functions. Decisive signaling pathways, such as the AKT pathway, the NOTCH pathway, and the JAK/STAT pathway modify antigen-induced T cell activation and emerge as promising therapeutic targets to halt disease progression and, eventually, reset the immune system to reestablish the immune privilege of the arterial wall.
Topics: Animals; Giant Cell Arteritis; Humans; Signal Transduction; Takayasu Arteritis
PubMed: 33072134
DOI: 10.3389/fimmu.2020.587089 -
Techniques in Vascular and... Dec 2014Large vessel vasculitis is very uncommon, particularly in Western countries, but can cause serious sequelae. Large vessel vasculitis is usually due to either Takayasu... (Review)
Review
Large vessel vasculitis is very uncommon, particularly in Western countries, but can cause serious sequelae. Large vessel vasculitis is usually due to either Takayasu arteritis or giant cell arteritis. The available laboratory tests are nonspecific, so the diagnosis is often dependent on imaging findings. The location and pattern of vessel narrowing will usually define the type of vasculitis. Symptomatic vascular stenoses and occlusions can frequently be managed using balloon angioplasty. Familiarity with the indications for treatment and techniques and outcomes associated with angioplasty and surgical alternatives is essential when treating these rare disorders.
Topics: Adult; Aged; Arteritis; Endovascular Procedures; Female; Humans; Middle Aged
PubMed: 25770639
DOI: 10.1053/j.tvir.2014.11.006 -
Presse Medicale (Paris, France : 1983) Sep 2019
Topics: Adult; Giant Cell Arteritis; Humans
PubMed: 31564552
DOI: 10.1016/j.lpm.2019.09.012 -
La Revue Du Praticien Apr 2023TREATMENT OF GIANT CELL ARTERITIS. The treatment of giant cell arteritis (GCA) is based on glucocorticoids. This treatment significantly reduces the risk of ischemic...
TREATMENT OF GIANT CELL ARTERITIS. The treatment of giant cell arteritis (GCA) is based on glucocorticoids. This treatment significantly reduces the risk of ischemic complications, especially those of a visual nature, rapidly relieves the symptoms of the disease, and eliminates the inflammatory syndrome. The diagnosis of GCA must be able to question if corticosteroid therapy is ineffective. Once the symptoms have resolved and the inflammatory syndrome has normalized, glucocorticosteroids are tapered very gradually. The goal is to discontinue glucocorticosteroids in 12 to 18 months. Nearly half of patients experience flares during the glucocorticoid taper. These are usually benign, not visually life-threatening, and easily controlled by increasing glucocorticoids. However, these relapses contribute to prolonging the treatment duration and thus the cumulative dose of glucocorticoids received by patients, which leads to the occurrence of adverse effects of glucocorticoids in almost all patients. For this reason, it is sometimes necessary to prescribe glucocorticoid-sparing treatments, particularly methotrexate and tocilizumab. The efficacy of these treatments and others in development is essential and to be discussed. In addition, the management of patients with GCA should include preventive measures to reduce cardiovascular, infectious and osteoporosis risks.
Topics: Humans; Glucocorticoids; Giant Cell Arteritis; Methotrexate
PubMed: 37289152
DOI: No ID Found -
BMJ Case Reports May 2021A young pregnant woman, G2P1L1, was admitted for safe confinement at 40 weeks of gestation with Takayasu arteritis. She was diagnosed with Takayasu arteritis in 2016...
A young pregnant woman, G2P1L1, was admitted for safe confinement at 40 weeks of gestation with Takayasu arteritis. She was diagnosed with Takayasu arteritis in 2016 when she had polyarthritis, hypertension and asymmetrical peripheral pulses. Her CT angiogram showed involvement of branches of aortic arch and coeliac trunk. She had mild pulmonary hypertension and was classified as type V disease (P)+. She was started on immunosuppressant medication and achieved a fair control of symptoms and disease activity. She gave history of treatment for pulmonary tuberculosis for 6 months in 2016 after which she developed polyarthralgia. She is currently asymptomatic and had mild hypertension that was controlled. She was evaluated for evidence of aneurysms/thrombus/aortic insufficiency and taken up for elective caesarean in view of type V disease. Maternal and perinatal outcome was good and she was discharged on her regular medication as per immunology opinion.
Topics: Angiography; Aorta, Thoracic; Female; Humans; Hypertension, Pulmonary; Pregnancy; Pregnancy Outcome; Takayasu Arteritis
PubMed: 34039540
DOI: 10.1136/bcr-2020-238014 -
Orphanet Journal of Rare Diseases Jul 2021The aim of this National Diagnostic and Care Protocol (PNDS) is to explain to the professionals involved the current optimal diagnosis and therapeutic management and... (Review)
Review
The aim of this National Diagnostic and Care Protocol (PNDS) is to explain to the professionals involved the current optimal diagnosis and therapeutic management and care approach for a patient with Takayasu's arteritis. Its purpose is to optimize and harmonize the management and follow-up of this rare disease throughout the country. It also identifies pharmaceutical specialties used in an indication not provided for in the Marketing Authorization, as well as the specialties, products or services necessary for the care of patients but not usually paid for or reimbursed.
Topics: Humans; Takayasu Arteritis
PubMed: 34284801
DOI: 10.1186/s13023-021-01922-1 -
Seminars in Neurology Dec 2019Giant cell arteritis (GCA) is the most common vasculitis in individuals 50 years or older, with a lifetime risk in the United States of 1% in women and 0.5% in men. It... (Review)
Review
Giant cell arteritis (GCA) is the most common vasculitis in individuals 50 years or older, with a lifetime risk in the United States of 1% in women and 0.5% in men. It is a granulomatous disease that affects large- and medium-sized vessels, most notably the branches of the internal and external carotid arteries. GCA can cause both afferent and efferent visual manifestations, the most common being arteritic anterior ischemic optic neuropathy. Diagnosis of GCA is made using criteria developed by the American College of Rheumatology, which include clinical signs, positive biopsy, and elevated erythrocyte sedimentation rate. C-reactive protein and platelet counts may be elevated in GCA, and noninvasive imaging modalities such as Doppler ultrasound and magnetic resonance imaging are now being used to aid in diagnosis. While glucocorticoids are the mainstay of treatment for GCA, new breakthrough treatments such as tocilizumab (an anti-IL-6 receptor antibody) have shown great promise in causing disease remission and reducing the cumulative glucocorticoid dose. Emerging therapies such as abatacept and ustekinumab are still being studied and may be of use to clinicians in the future.
Topics: Giant Cell Arteritis; Humans; Optic Neuropathy, Ischemic
PubMed: 31847038
DOI: 10.1055/s-0039-1698761 -
La Revue de Medecine Interne Apr 2016Aortitis is a frequent complication of giant cell arteritis. Imaging techniques can reveal the inflammation of the aortic wall. CT-scan can show circumferential aortic... (Review)
Review
Aortitis is a frequent complication of giant cell arteritis. Imaging techniques can reveal the inflammation of the aortic wall. CT-scan can show circumferential aortic wall thickening, or TEP-scan can show aortic FDG-uptake. Aortic aneurysm and dissection is a feared but probably rare complication of the inflammation of the aortic wall during GCA. Screening for aortitis could be proposed for patients with symptoms of aortic involvement, for patients with signs of large vessels involvement (limb claudication, bruit) or for patients with incomplete response to treatment. The best follow-up and treatment are to be determined for the patients with aortitis related to GCA.
Topics: Aortitis; Diagnostic Imaging; Giant Cell Arteritis; Humans; Incidence
PubMed: 26781692
DOI: 10.1016/j.revmed.2015.12.018 -
Journal of Medical Case Reports Jul 2023Takayasu arteritis is a rare and chronic granulomatous vasculitis that affects the large vessels. Takayasu arteritis targets the aorta and its branches and is still of...
BACKGROUND
Takayasu arteritis is a rare and chronic granulomatous vasculitis that affects the large vessels. Takayasu arteritis targets the aorta and its branches and is still of unknown etiology. It often affects female patients under 50 years of age. A relationship between Takayasu arteritis and tuberculosis has been suggested for a long time.
CASE PRESENTATION
We report a severe case of Takayasu arteritis in a 10-year-old Tunisian child revealed by renovascular hypertension with concomitant pulmonary tuberculosis.
CONCLUSIONS
Our patient is among only a few cases of Takayasu arteritis published worldwide affecting young infants and adolescents, which underlines the strong relationship between Takayasu arteritis and tuberculosis.
Topics: Child; Adolescent; Humans; Female; Takayasu Arteritis; Aorta; Tuberculosis
PubMed: 37455309
DOI: 10.1186/s13256-023-04037-2