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Best Practice & Research. Clinical... Feb 2018Vascular surgery remains an important option in the management of Takayasu arteritis (TA). Its use is predominantly confined to the treatment of symptomatic organ... (Review)
Review
Vascular surgery remains an important option in the management of Takayasu arteritis (TA). Its use is predominantly confined to the treatment of symptomatic organ ischaemia or life-threatening aneurysm formation. In most cases, this follows the failure of medical therapy to prevent arterial injury. Open surgery and endovascular approaches are used. The choice between them, at least in part, is determined by the site and nature of the lesion. Open surgery, although more invasive, offers enhanced duration of arterial patency, whereas for endovascular intervention, primary angioplasty without stenting is preferred, with stenting reserved for primary or secondary angioplasty failures. Although there is increasing interest in the role of stent grafts and tailor-made endovascular stents, long-term outcomes remain to be reported. Interventional outcomes are improved and complications reduced by therapeutic control of disease activity before and after surgery. The wider use of combined immunosuppression and the introduction of biologic therapy for refractory TA may reduce future requirements for surgical intervention.
Topics: Angioplasty; Humans; Takayasu Arteritis; Treatment Outcome
PubMed: 30526891
DOI: 10.1016/j.berh.2018.07.008 -
Giant Cell Arteritis: Advances in Understanding Pathogenesis and Implications for Clinical Practice.Cells Jan 2024Giant cell arteritis (GCA) is a noninfectious granulomatous vasculitis of unknown etiology affecting individuals older than 50 years. Two forms of GCA have been... (Review)
Review
Giant cell arteritis (GCA) is a noninfectious granulomatous vasculitis of unknown etiology affecting individuals older than 50 years. Two forms of GCA have been identified: a cranial form involving the medium-caliber temporal artery causing temporal arteritis (TA) and an extracranial form involving the large vessels, mainly the thoracic aorta and its branches. GCA generally affects individuals with a genetic predisposition, but several epigenetic (micro)environmental factors are often critical for the onset of this vasculitis. A key role in the pathogenesis of GCA is played by cells of both the innate and adaptive immune systems, which contribute to the formation of granulomas that may include giant cells, a hallmark of the disease, and arterial tertiary follicular organs. Cells of the vessel wall cells, including vascular smooth muscle cells (VSMCs) and endothelial cells, actively contribute to vascular remodeling responsible for vascular stenosis and ischemic complications. This review will discuss new insights into the molecular and cellular pathogenetic mechanisms of GCA, as well as the implications of these findings for the development of new diagnostic biomarkers and targeted drugs that could hopefully replace glucocorticoids (GCs), still the backbone of therapy for this vasculitis.
Topics: Humans; Giant Cell Arteritis; Endothelial Cells; Glucocorticoids
PubMed: 38334659
DOI: 10.3390/cells13030267 -
Der Nervenarzt Aug 2022Giant cell arteritis (GCA) is the most common idiopathic systemic vasculitis in the age group over 50 years. It requires prompt diagnostics and treatment to avoid severe... (Review)
Review
Giant cell arteritis (GCA) is the most common idiopathic systemic vasculitis in the age group over 50 years. It requires prompt diagnostics and treatment to avoid severe complications, such as visual loss or stroke. The tendency to relapse makes a glucocorticoid (GC) treatment necessary for several years and sometimes lifelong, which increases the risk of GC-induced long-term side effects. Therefore, additive GC-sparing treatment is recommended in the majority of patients. For this purpose, the anti-IL‑6 receptor antibody tocilizumab is available as an approved substance for subcutaneous application; alternatively, methotrexate (MTX) can be used (off-label).
Topics: Drug Therapy, Combination; Giant Cell Arteritis; Glucocorticoids; Humans; Methotrexate; Middle Aged; Recurrence
PubMed: 34734295
DOI: 10.1007/s00115-021-01216-8 -
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 -
Current Rheumatology Reports Feb 2021Large vessel vasculitides (LVVs) are inflammatory conditions of the wall of large-sized arteries, mainly represented by giant cell arteritis (GCA) and Takayasu arteritis... (Review)
Review
PURPOSE OF REVIEW
Large vessel vasculitides (LVVs) are inflammatory conditions of the wall of large-sized arteries, mainly represented by giant cell arteritis (GCA) and Takayasu arteritis (TA). The inflammatory process within the vessel wall can lead to serious consequences such as development of aneurysms, strokes and blindness; therefore, early diagnosis and follow-up of LVV are fundamental. However, the arterial wall is poorly accessible and blood biomarkers are intended to help physicians not only in disease diagnosis but also in monitoring and defining the prognosis of these conditions, thus assisting therapeutic decisions and favouring personalised management. The field is the object of intense research as the identification of reliable biomarkers is likely to shed light on the mechanisms of disease progression and arterial remodelling. In this review, we will discuss the role of blood biomarkers in LVVs in the light of the latest evidence.
RECENT FINDINGS
In clinical practice, the most widely performed laboratory investigations are the erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP). However, these indices may be within normal limits during disease relapse and they are not reliable in patients receiving interleukin-6 (IL-6) receptor inhibitors. New biomarkers struggle to gain traction in clinical practice and no molecule with good accuracy has been identified to date. IL-6, a pro-inflammatory cytokine that drives CRP synthesis and increases the ESR, is one of the most promising biomarkers in the field. IL-6 analysis is increasingly performed, and serum levels are more sensitive than ESR for active GCA and might reflect persistent inflammation with high risk of relapse in patients on IL-6 receptor inhibitors. A future with biomarkers that reflect different disease features is an important aspiration. Accordingly, intense effort is being made to identify IL-6-independent inflammatory biomarkers, such as S100 proteins, pentraxin-3 and osteopontin. Moreover, metalloproteinases such as MMP2/9 and angiogenic modulators such as VEGF, YLK-40 and angiopoietins are being studied as markers of arterial remodelling. Lastly, biomarkers indicating organ damage may guide prognostic stratification as well as emergency therapeutic decisions: the most promising biomarkers so far identified are NT-proBNP, which reflects myocardial strain; pentraxin-3, which has been associated with recent optic nerve ischemia; and endothelin-1, which is associated with ischaemic complications. Currently, the use of these molecules in clinical practice is limited because of their restricted availability, lack of sufficient studies supporting their validity and associated costs. Further evidence is required to better interpret their biological and clinical value.
Topics: Biomarkers; Cytokines; Giant Cell Arteritis; Humans; Prognosis; Takayasu Arteritis; Vasculitis
PubMed: 33569633
DOI: 10.1007/s11926-021-00980-5 -
Archives of Pathology & Laboratory... Sep 2017Giant cell arteritis is an inflammatory lesion of the large- and medium-sized arteries, usually involving the temporal arteries of older women. Rarely, the breast has... (Review)
Review
Giant cell arteritis is an inflammatory lesion of the large- and medium-sized arteries, usually involving the temporal arteries of older women. Rarely, the breast has been reported as the primary site of involvement. Patients usually present with breast masses that are tender or painful in most reported cases. The disease may be associated with constitutional symptoms that resemble polymyalgia rheumatica, but most reported cases do not have an established diagnosis of autoimmune disease. Clinically, giant cell arteritis of the breast may mimic breast carcinoma. Establishing the diagnosis is important because most patients achieve remission of symptoms after treatment with prednisone.
Topics: Breast Diseases; Breast Neoplasms; Diagnosis, Differential; Female; Giant Cell Arteritis; Humans
PubMed: 28854344
DOI: 10.5858/arpa.2016-0285-RS -
Current Pain and Headache Reports Oct 2022Giant cell arteritis (GCA) is a chronic, inflammatory condition, primarily affecting the medium and larger arteries. The purpose of this narrative review is to describe... (Review)
Review
PURPOSE OF REVIEW
Giant cell arteritis (GCA) is a chronic, inflammatory condition, primarily affecting the medium and larger arteries. The purpose of this narrative review is to describe GCA in the context of headache and facial pain, based on a case and the available current literature. Understanding the etiology, pathophysiology, the associated conditions, and the differential diagnoses is important in managing GCA.
RECENT FINDINGS
In a patient presenting with unilateral facial/head pain with disturbances of vision, GCA should be considered in the differential diagnosis. There is an association of GCA with several comorbid conditions, and infections including coronavirus-19 (COVID-19) infection. Management of GCA primarily depends upon the identification of the affected artery and prompt treatment. Permanent visual loss and other serious complications are associated with GCA. GCA is characterized by robust inflammation of large- and medium-sized arteries and marked elevation of systemic mediators of inflammation. An interdisciplinary approach of management involving the pertinent specialties is strongly recommended.
Topics: Humans; Giant Cell Arteritis; Temporal Arteries; COVID-19; Facial Pain; Headache; Chronic Disease; Inflammation Mediators
PubMed: 36057073
DOI: 10.1007/s11916-022-01075-1 -
Eye (London, England) Aug 2023Giant Cell Arteritis (GCA) is well known to be a critical ischaemic disease that requires immediate medical recognition to initiate treatment and where one in five... (Review)
Review
Giant Cell Arteritis (GCA) is well known to be a critical ischaemic disease that requires immediate medical recognition to initiate treatment and where one in five people still suffer visual loss. The immunopathophysiology has continued to be characterised, and the influencing of ageing in the development of GCA is beginning to be understood. Recent national and international guidelines have supported the directed use of cranial ultrasound to reduce diagnostic delay and improve clinical outcomes. Immediate high dose glucocorticoids remain the standard emergency treatment for GCA, with a number of targeted agents that have been shown in clinical trials to have superior clinical efficacy and steroid sparing effects. The aim of this review was to present the latest advances in GCA that have the potential to influence routine clinical practice.
Topics: Humans; Giant Cell Arteritis; Delayed Diagnosis; Glucocorticoids; Aging; Treatment Outcome
PubMed: 36788362
DOI: 10.1038/s41433-023-02433-y -
Indian Journal of Ophthalmology Oct 2023Giant cell arteritis (GCA) is a granulomatous inflammation involving medium and large vessels that can lead to serious clinical manifestations associated with tissue... (Review)
Review
Giant cell arteritis (GCA) is a granulomatous inflammation involving medium and large vessels that can lead to serious clinical manifestations associated with tissue ischemia. Temporal artery biopsy (TAB) is currently the gold standard method for the diagnosis of GCA, with a specificity of 100% and a sensitivity of 77%. However, the false-negative rate for TAB ranges from 9% to 61%. False negatives may be related to the timing of biopsy, the length of specimen, and the existence of "skip lesions." We reviewed the relevant evidence for methods to improve the sensitivity and reduce the false-negative rate for TAB. To reduce the false-negative rate for TAB, it is recommended to perform TAB within 1 week of starting corticosteroid therapy. Although there is currently no consensus, we suggest that the temporal artery is cut to a length of 20‒30 mm and to prepare serial pathological sections. It is necessary to attach great importance to patients suspected of having GCA, and complete TAB should be performed as soon as possible while starting corticosteroid therapy promptly. We also discuss the clinical value of non-invasive vascular imaging technologies, such as DUS, CTA, MRA, and 18F-FDG-PET/CT, as auxiliary methods for GCA diagnosis that could partially replace TAB.
Topics: Humans; Giant Cell Arteritis; Temporal Arteries; Positron Emission Tomography Computed Tomography; Sensitivity and Specificity; Biopsy; Adrenal Cortex Hormones; Retrospective Studies
PubMed: 37787225
DOI: 10.4103/IJO.IJO_3163_22 -
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