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Turkish Journal of Medical Sciences Aug 2018Takayasu arteritis (TAK) is a challenging chronic, granulomatous, large-vessel systemic vasculitis, mostly due to difficulties in early diagnosis and assessing actual... (Review)
Review
Takayasu arteritis (TAK) is a challenging chronic, granulomatous, large-vessel systemic vasculitis, mostly due to difficulties in early diagnosis and assessing actual disease activity. Since there are no specific diagnostic laboratory tests, biomarkers, or autoantibodies, many patients experience considerable delay in diagnosis. Remembering the possibility of TAK together with the use of acute phase responses and appropriate imaging studies may be helpful for early diagnosis. Since there may be discrepancies between systemic and vascular wall inflammation, using only acute phase responses is not reliable in assessing current disease activity. Therefore, physical examination and new imaging findings should also be used to assess current disease activity. Despite its limitations, the Indian Takayasu Clinical Activity Score (ITAS2010) may also be helpful for this purpose. The rationale of medical treatment is to suppress both vascular and systemic inflammation with appropriate systemic immunosuppression, including corticosteroids and conventional immunosuppressive agents. In cases of refractory disease activity, leflunomide and biologic agents such as TNF inhibitors and tocilizumab may be tried. In selected cases with persistent lesions that cannot be reversed with medical treatment, endovascular interventions including balloon angioplasty, stent and stent graft replacement, or surgery may be tried. However, such procedures should be performed after suppression of inflammation, i.e. during inactive disease. Prognosis of TAK is probably getting better with lower mortality rates reported in recent years, probably due to the use of more effective medical treatments as well as the use of endovascular interventions when necessary and available.
Topics: Antibodies, Monoclonal, Humanized; Biomarkers; Endovascular Procedures; Guidelines as Topic; Humans; Immunosuppressive Agents; Leflunomide; Physical Examination; Prognosis; Radiology, Interventional; Takayasu Arteritis
PubMed: 30114347
DOI: 10.3906/sag-1804-136 -
Respiratory Medicine Dec 2018The hallmark of vasculitis is autoimmune inflammation of blood vessels and surrounding tissues, resulting in an array of constitutional symptoms and organ damage. The... (Review)
Review
The hallmark of vasculitis is autoimmune inflammation of blood vessels and surrounding tissues, resulting in an array of constitutional symptoms and organ damage. The lung is commonly targeted in the more familiar ANCA-associated small vessel vasculitidies, but large and medium vessel vasculitides, including Takayasu arteritis, giant cell arteritis, polyarteritis nodosa, Behcet's disease, and necrotizing sarcoid granulomatosis, may also feature prominent pulmonary involvement. Pulmonary manifestations of these conditions include pulmonary arterial aneurysms, pulmonary hypertension, diffuse alveolar hemorrhage, pulmonary nodules, and parenchymal infiltrates. An understanding of the diverse manifestations of vasculitis and a high index of clinical suspicion are essential to avoid delays in disease recognition that may result in permanent or life threatening morbidity. In this review, we outline the general clinical manifestations, pulmonary manifestations, diagnostic workup, imaging findings, and treatment of medium, large, and variable vessel vasculitides.
Topics: Aneurysm; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis; Behcet Syndrome; Giant Cell Arteritis; Hemorrhage; Humans; Hypertension, Pulmonary; Lung Diseases; Polyarteritis Nodosa; Pulmonary Alveoli; Pulmonary Artery; Takayasu Arteritis
PubMed: 30509707
DOI: 10.1016/j.rmed.2018.11.003 -
The New England Journal of Medicine Oct 2022
Topics: Humans; Giant Cell Arteritis; Temporal Arteries
PubMed: 36214594
DOI: 10.1056/NEJMicm2202567 -
Rheumatology (Oxford, England) Feb 2019Although outcomes in Takayasu arteritis (TAK) are improving, diagnosis is typically delayed and significant arterial injury accrues. While wider use of non-invasive... (Review)
Review
Although outcomes in Takayasu arteritis (TAK) are improving, diagnosis is typically delayed and significant arterial injury accrues. While wider use of non-invasive imaging is impacting this, the onus remains with clinicians to consider a diagnosis of TAK earlier. Meanwhile, morbidity and mortality in TAK remains increased. Herein we review the current situation, outline recent advances and summarize remaining challenges. Understanding of disease pathogenesis remains poor. However, recent genetic data and identification of pathogenic cytokines may facilitate the search for biomarkers capable of distinguishing active and inactive disease, inflammatory and non-inflammatory arterial remodelling. Imaging is critical for TAK, and each modality has important strengths and limitations. Dependence upon CS therapy remains too high. However, the impact of combination immunosuppressive therapy is now recognized, biologic therapies are increasingly available and new agents offer promise. Multicentre clinical trials are now required, and these will depend upon development of defined clinical and imaging end-points.
Topics: Antirheumatic Agents; Biological Products; Biomarkers; Computed Tomography Angiography; Disease Progression; Genetic Predisposition to Disease; Humans; Immunosuppressive Agents; Magnetic Resonance Angiography; Severity of Illness Index; Takayasu Arteritis
PubMed: 29635396
DOI: 10.1093/rheumatology/key040 -
Presse Medicale (Paris, France : 1983) Apr 2020Aortitis and periaortitis are inflammatory diseases of the aorta and its main branches; they differ in the extension of inflammation, which is confined to the aortic... (Review)
Review
Aortitis and periaortitis are inflammatory diseases of the aorta and its main branches; they differ in the extension of inflammation, which is confined to the aortic wall in aortitis, and spreads to the periaortic space in periaortitis. Aortitis is classified as non-infectious or infectious. Non-infectious aortitis represents a common feature of large-vessel vasculitides but can also be isolated or associated with other rheumatologic conditions. Periaortitis can be idiopathic or secondary to a wide array of etiologies such as drugs, infections, malignancies, and other proliferative diseases. Notably, both aortitis and periaortitis may arise in the context of IgG4-related disease, a recently characterised fibro-inflammatory systemic disease. Prompt recognition, correct diagnosis and appropriate treatment are essential in order to avoid life-threatening complications.
Topics: Aorta; Aortitis; Diagnostic Imaging; Giant Cell Arteritis; Humans; Immunoglobulin G4-Related Disease; Retroperitoneal Fibrosis; Takayasu Arteritis
PubMed: 32234379
DOI: 10.1016/j.lpm.2020.104018 -
La Revue Du Praticien Apr 2023TAKAYASU'S ARTERITIS. Takayasu's arteritis is an inflammatory panarteritis of the large vessels, preferentially affecting the aorta, its main branches, and the pulmonary...
TAKAYASU'S ARTERITIS. Takayasu's arteritis is an inflammatory panarteritis of the large vessels, preferentially affecting the aorta, its main branches, and the pulmonary arteries. Its incidence is estimated at 1.11 cases per million person-years, with a female predominance. The disease is classically characterized by the succession of two phases: a pre-occlusive inflammatory phase that may go unnoticed and an occlusive phase characterized by ischemic vascular symptoms because of parietal arterial lesions such as stenosis, occlusion or aneurysm. The diagnosis is based on clinical, biological and morphological findings. When available, pathological examination reveals a predominantly medial-adventitial, segmental and focal granulomatous panarteritis. Treatment consists of administering corticosteroid therapy and often immunosuppressants, or even biotherapies, managing cardiovascular risk factors, and managing vascular complications.
Topics: Humans; Female; Male; Takayasu Arteritis
PubMed: 37289153
DOI: No ID Found -
Ugeskrift For Laeger Nov 2023Cranial and large vessel giant cell arteritis is a systemic vasculitis frequently found in Denmark. In recent years, considerable new knowledge has been gained in the... (Review)
Review
Cranial and large vessel giant cell arteritis is a systemic vasculitis frequently found in Denmark. In recent years, considerable new knowledge has been gained in the field of diagnostics and treatment of giant cell arteritis. In this review, the clinical and radiological findings, treatment options and effect and side effects of treatment with tocilizumab are reviewed in a Danish context.
Topics: Humans; Giant Cell Arteritis
PubMed: 37987451
DOI: No ID Found -
Current Opinion in Allergy and Clinical... Aug 2023Giant cell arteritis (GCA) is an idiopathic and persistent condition characterized by granulomatous vasculitis of the medium and large vessels with overlapping... (Review)
Review
PURPOSE OF REVIEW
Giant cell arteritis (GCA) is an idiopathic and persistent condition characterized by granulomatous vasculitis of the medium and large vessels with overlapping phenotypes, including conventional cranial arteritis and extra-cranial GCA, also known as large-vessel GCA. Vascular problems linked with large vessel involvement may partly be caused by delayed diagnosis, emphasizing the necessity of early detection and the fast beginning of appropriate therapy. Glucocorticoids are the cornerstone of treatment for GCA, but using them for an extended period has numerous, often severe, side effects.
RECENT FINDINGS
clinical practice and novel discoveries on the pathogenic pathways suggest that steroid-free biologic treatments may be efficient and safe for GCA patients.
SUMMARY
since now, only Tocilizumab is approved for GCA treatment, but several drugs are currently used, and ongoing trials could give both researchers and patients novel therapeutic strategies for induction, maintenance, and prevention of relapse of GCA. The aims of this work is to synthesize evidence from current studies present in scientific literature about innovative treatment of Giant cell artheritis.
Topics: Humans; Giant Cell Arteritis; Glucocorticoids
PubMed: 37357797
DOI: 10.1097/ACI.0000000000000923 -
Rheumatic Diseases Clinics of North... Aug 2023Predictions for a general path forward in vasculitis care and research are provided based on advances made in the past 20 years. Prospects for advances in translational... (Review)
Review
Predictions for a general path forward in vasculitis care and research are provided based on advances made in the past 20 years. Prospects for advances in translational research with potential to improve care are highlighted, including identification of hemato-inflammatory diseases, autoantigens, disease mechanisms in animal models, and biomarkers. A list of active randomized trials is provided, and areas of potential paradigm shifts in care are highlighted. The importance of patient involvement and international collaboration is noted, and a plea is made for innovative trial designs that would improve access of patients to trials and to clinical experts at referral centers.
Topics: Animals; Humans; Takayasu Arteritis; Biomarkers; Forecasting; Giant Cell Arteritis
PubMed: 37331742
DOI: 10.1016/j.rdc.2023.03.014 -
The Lancet. Rheumatology Jun 2024Giant cell arteritis is the principal form of systemic vasculitis affecting people over 50. Large-vessel involvement, termed large vessel giant cell arteritis, mainly... (Review)
Review
Giant cell arteritis is the principal form of systemic vasculitis affecting people over 50. Large-vessel involvement, termed large vessel giant cell arteritis, mainly affects the aorta and its branches, often occurring alongside cranial giant cell arteritis, but large vessel giant cell arteritis without cranial giant cell arteritis can also occur. Patients mostly present with constitutional symptoms, with localising large vessel giant cell arteritis symptoms present in a minority of patients only. Large vessel giant cell arteritis is usually overlooked until clinicians seek to exclude it with imaging by ultrasonography, magnetic resonance angiography (MRA), computed tomography angiography (CTA), or [F]fluorodeoxyglucose-PET-CT. Although the role of imaging in treatment monitoring remains uncertain, imaging by MRA or CTA is crucial for identifying aortic aneurysm formation during patient follow up. In this Series paper, we define the large vessel subset of giant cell arteritis and summarise its clinical challenges. Furthermore, we identify areas for future research regarding the management of large vessel giant cell arteritis.
Topics: Giant Cell Arteritis; Humans; Positron Emission Tomography Computed Tomography; Magnetic Resonance Angiography; Computed Tomography Angiography
PubMed: 38574745
DOI: 10.1016/S2665-9913(23)00300-4