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Nature Reviews. Disease Primers Jan 2022Large-vessel vasculitis (LVV) manifests as inflammation of the aorta and its major branches and is the most common primary vasculitis in adults. LVV comprises two... (Review)
Review
Large-vessel vasculitis (LVV) manifests as inflammation of the aorta and its major branches and is the most common primary vasculitis in adults. LVV comprises two distinct conditions, giant cell arteritis and Takayasu arteritis, although the phenotypic spectrum of primary LVV is complex. Non-specific symptoms often predominate and so patients with LVV present to a range of health-care providers and settings. Rapid diagnosis, specialist referral and early treatment are key to good patient outcomes. Unfortunately, disease relapse remains common and chronic vascular complications are a source of considerable morbidity. Although accurate monitoring of disease activity is challenging, progress in vascular imaging techniques and the measurement of laboratory biomarkers may facilitate better matching of treatment intensity with disease activity. Further, advances in our understanding of disease pathophysiology have paved the way for novel biologic treatments that target important mediators of disease in both giant cell arteritis and Takayasu arteritis. This work has highlighted the substantial heterogeneity present within LVV and the importance of an individualized therapeutic approach. Future work will focus on understanding the mechanisms of persisting vascular inflammation, which will inform the development of increasingly sophisticated imaging technologies. Together, these will enable better disease prognostication, limit treatment-associated adverse effects, and facilitate targeted development and use of novel therapies.
Topics: Adult; Aorta; Giant Cell Arteritis; Humans; Takayasu Arteritis
PubMed: 34992251
DOI: 10.1038/s41572-021-00327-5 -
Arthritis & Rheumatology (Hoboken, N.J.) Aug 2021To provide evidence-based recommendations and expert guidance for the management of giant cell arteritis (GCA) and Takayasu arteritis (TAK) as exemplars of large vessel...
OBJECTIVE
To provide evidence-based recommendations and expert guidance for the management of giant cell arteritis (GCA) and Takayasu arteritis (TAK) as exemplars of large vessel vasculitis.
METHODS
Clinical questions regarding diagnostic testing, treatment, and management were developed in the population, intervention, comparator, and outcome (PICO) format for GCA and TAK (27 for GCA, 27 for TAK). Systematic literature reviews were conducted for each PICO question. The Grading of Recommendations Assessment, Development and Evaluation methodology was used to rate the quality of the evidence. Recommendations were developed by the Voting Panel, comprising adult and pediatric rheumatologists and patients. Each recommendation required ≥70% consensus among the Voting Panel.
RESULTS
We present 22 recommendations and 2 ungraded position statements for GCA, and 20 recommendations and 1 ungraded position statement for TAK. These recommendations and statements address clinical questions relating to the use of diagnostic testing, including imaging, treatments, and surgical interventions in GCA and TAK. Recommendations for GCA include support for the use of glucocorticoid-sparing immunosuppressive agents and the use of imaging to identify large vessel involvement. Recommendations for TAK include the use of nonglucocorticoid immunosuppressive agents with glucocorticoids as initial therapy. There were only 2 strong recommendations; the remaining recommendations were conditional due to the low quality of evidence available for most PICO questions.
CONCLUSION
These recommendations provide guidance regarding the evaluation and management of patients with GCA and TAK, including diagnostic strategies, use of pharmacologic agents, and surgical interventions.
Topics: Disease Management; Evidence-Based Medicine; Giant Cell Arteritis; Humans; Immunosuppressive Agents; Rheumatology; Takayasu Arteritis; United States
PubMed: 34235884
DOI: 10.1002/art.41774 -
Postgraduate Medicine Jan 2023Takayasu's arteritis is a rare, chronic, large vessel vasculitis which affects the aorta and its large branches. Early diagnosis is important to prevent serious end... (Review)
Review
Takayasu's arteritis is a rare, chronic, large vessel vasculitis which affects the aorta and its large branches. Early diagnosis is important to prevent serious end organ damage, including to stroke and ischemic heart disease. Studies have demonstrated treatment response with methotrexate, azathioprine, and tumor necrosis factor (TNF) inhibitors. This article aims to describe to the reader key features of Takayasu's arteritis and highlights updated evidence in the diagnosis and management of these patients. We also review the 2021 ACR guidelines for Takayasu's arteritis and correlate them to the updated evidence discussed throughout the review. An extensive literature search was conducted via PUBMED, including the words 'Takayasu's diagnostic criteria,' 'Takayasu's treatment,' 'Takayasu's diagnosis,' 'Takayasu's imaging', and 'Takayasu's diagnostic criteria.' Search was filtered to include clinical trials, randomized controlled trials, systematic reviews, and meta-analyses. Articles in the English language or with English translation and published by the date of 20 December 2021 were included.
Topics: Humans; Takayasu Arteritis; Azathioprine
PubMed: 36588528
DOI: 10.1080/00325481.2022.2159723 -
Circulation Jan 2021Systemic vasculitides are multisystem blood vessel disorders, which are defined by the size of the vessel predominantly affected, namely small, medium, or large vessels.... (Review)
Review
Systemic vasculitides are multisystem blood vessel disorders, which are defined by the size of the vessel predominantly affected, namely small, medium, or large vessels. The term "large vessel" relates to the aorta and its major branches; "medium vessel" refers to the main visceral arteries and veins and their initial branches. The most common causes of large-vessel vasculitis are giant cell arteritis and Takayasu arteritis, and those of medium-vessel arteritis are polyarteritis nodosa and Kawasaki disease. However, there is some overlap, and arteries of any size can potentially be involved in any of the 3 main categories of dominant vessel involvement. In addition to multisystem vasculitides, other forms of vasculitis have been defined, including single-organ vasculitis (eg, isolated aortitis). Prompt identification of vasculitides is important because they are associated with an increased risk of mortality. Left undiagnosed or mismanaged, these conditions may result in serious adverse outcomes that might otherwise have been avoided or minimized. The ethnic and regional differences in the incidence, prevalence, and clinical characteristics of patients with vasculitis should be recognized. Because the clinical presentation of vasculitis is highly variable, the cardiovascular clinician must have a high index of suspicion to establish a reliable and prompt diagnosis. This article reviews the pathophysiology, epidemiology, diagnostic strategies, and management of vasculitis.
Topics: Animals; Giant Cell Arteritis; Glucocorticoids; Humans; Mucocutaneous Lymph Node Syndrome; Polyarteritis Nodosa; Takayasu Arteritis; Vasculitis
PubMed: 33464968
DOI: 10.1161/CIRCULATIONAHA.120.046657 -
Neurologic Clinics May 2019"Giant cell arteritis (GCA) is a chronic, idiopathic, granulomatous vasculitis of medium and large arteries comprising overlapping phenotypes of cranial arteritis and... (Review)
Review
"Giant cell arteritis (GCA) is a chronic, idiopathic, granulomatous vasculitis of medium and large arteries comprising overlapping phenotypes of cranial arteritis and extracranial GCA. Vascular complications are generally due to delay in diagnosis and initiation of effective treatment. Advancements in MRI and MR angiography, computed tomography angiography, 18fluoro-deoxyglucose/PET, and color duplex ultrasonography have led to improved diagnosis. Corticosteroids are the mainstay of therapy in GCA; however, their use is associated with predictable and occasionally serious side effects. Biological agents are effective and safe corticosteroid-sparing agents in treating GCA. This article reviews the epidemiologic, clinicopathologic features, diagnosis, and treatment of GCA."
Topics: Giant Cell Arteritis; Humans
PubMed: 30952412
DOI: 10.1016/j.ncl.2019.01.008 -
Current Rheumatology Reviews 2019Giant Cell Arteritis (GCA), or Horton's Arteritis, is a chronic form of vasculitis of the large and medium vessels, especially involving the extracranial branches of the... (Review)
Review
Giant Cell Arteritis (GCA), or Horton's Arteritis, is a chronic form of vasculitis of the large and medium vessels, especially involving the extracranial branches of the carotid arteries, in particular, the temporal artery, with the involvement of the axillary, femoral and iliac arteries too. Arterial wall inflammation leads to luminal occlusion and tissue ischemia, which is responsible for the clinical manifestations of the disease. A substantial number of patients affected by GCA present head and neck symptoms, including ocular, neurological and otorhinolaryngological manifestations. The aim of this article is to present pathogenesis, clinical aspects and treatment approaches of GCA manifestations.
Topics: Adrenal Cortex Hormones; Giant Cell Arteritis; Humans; Otorhinolaryngologic Diseases; Prognosis
PubMed: 30827250
DOI: 10.2174/1573397115666190227194014 -
Postgraduate Medicine Jan 2023Giant cell arteritis (GCA, also known as temporal arteritis) is classified as a vasculitis of large and medium sized vessels and can involve the cranial vessels as well... (Review)
Review
Giant cell arteritis (GCA, also known as temporal arteritis) is classified as a vasculitis of large and medium sized vessels and can involve the cranial vessels as well as the aorta and great vessels. It is a systemic rheumatic disease that virtually never occurs in adults younger than 50 years of age. GCA is the most common idiopathic systemic vasculitis. Systemic symptoms are common and involvement of the muscular, extracranial branches off of the carotid arteries are what result in the classic manifestations of cranial GCA. The disease can also be generalized involving the aorta and its branches leading to aneurysms and stenosis of involved vessels. Glucocorticoids have been the longstanding treatment option for GCA but relatively recent studies have proven additional agents like Tocilizumab are effective steroid sparing agents. GCA is a disease that is of variable duration and length of treatment differs from patient to patient. This article will review the epidemiology, pathogenesis, clinical manifestations, work up and treatment options for GCA.
Topics: Humans; Giant Cell Arteritis; Glucocorticoids
PubMed: 37021621
DOI: 10.1080/00325481.2023.2190288 -
Current Rheumatology Reports Aug 2020Giant cell arteritis (GCA) and Takayasu arteritis (TAK) are auto-inflammatory and autoimmune diseases with a highly selective tissue tropism for medium and large... (Review)
Review
PURPOSE OF REVIEW
Giant cell arteritis (GCA) and Takayasu arteritis (TAK) are auto-inflammatory and autoimmune diseases with a highly selective tissue tropism for medium and large arteries. In both diseases, CD4 T cells and macrophages form granulomatous lesions within the arterial wall, a tissue site normally protected by immune privilege. Vascular lesions can be accompanied by an extravascular component, typically an intense hepatic acute phase response that produces well-known laboratory abnormalities, e.g., elevated ESR and CRP. It is unclear whether GCA and TAK lie on a spectrum of disease or whether they represent fundamentally different disease processes.
RECENT FINDINGS
GCA and TAK share many clinical features, but there are substantial differences in genetics, epidemiology, disease mechanisms, response to treatment, and treatment complications that give rise to different disease trajectories. A significant difference lies in the composition of the wall-infiltrating immune cell compartment, which in TAK includes a significant population of CD8 T cells as well as natural killer cells, specifying disparate disease effector pathways mediating tissue damage and vessel wall remodeling. Despite the similarities in tissue tropism and histomorphology, GCA and TAK are two distinct vasculitides that rely on separate disease mechanisms and require disease-specific approaches in diagnosis and management.
Topics: CD8-Positive T-Lymphocytes; Diagnosis, Differential; Giant Cell Arteritis; Humans; Killer Cells, Natural; Macrophages; Takayasu Arteritis
PubMed: 32845392
DOI: 10.1007/s11926-020-00948-x -
Annals of the Rheumatic Diseases Jan 2020Since the publication of the European League Against Rheumatism (EULAR) recommendations for the management of large vessel vasculitis (LVV) in 2009, several relevant...
BACKGROUND
Since the publication of the European League Against Rheumatism (EULAR) recommendations for the management of large vessel vasculitis (LVV) in 2009, several relevant randomised clinical trials and cohort analyses have been published, which have the potential to change clinical care and therefore supporting the need to update the original recommendations.
METHODS
Using EULAR standardised operating procedures for EULAR-endorsed recommendations, the EULAR task force undertook a systematic literature review and sought opinion from 20 experts from 13 countries. We modified existing recommendations and created new recommendations.
RESULTS
Three overarching principles and 10 recommendations were formulated. We recommend that a suspected diagnosis of LVV should be confirmed by imaging or histology. High dose glucocorticoid therapy (40-60 mg/day prednisone-equivalent) should be initiated immediately for induction of remission in active giant cell arteritis (GCA) or Takayasu arteritis (TAK). We recommend adjunctive therapy in selected patients with GCA (refractory or relapsing disease, presence of an increased risk for glucocorticoid-related adverse events or complications) using tocilizumab. Methotrexate may be used as an alternative. Non-biological glucocorticoid-sparing agents should be given in combination with glucocorticoids in all patients with TAK and biological agents may be used in refractory or relapsing patients. We no longer recommend the routine use of antiplatelet or anticoagulant therapy for treatment of LVV unless it is indicated for other reasons.
CONCLUSIONS
We have updated the recommendations for the management of LVV to facilitate the translation of current scientific evidence and expert opinion into better management and improved outcome of patients in clinical practice.
Topics: Antibodies, Monoclonal, Humanized; Antirheumatic Agents; Aortitis; Giant Cell Arteritis; Glucocorticoids; Humans; Methotrexate; Takayasu Arteritis
PubMed: 31270110
DOI: 10.1136/annrheumdis-2019-215672 -
Veterinary Journal (London, England :... 2023Steroid-responsive meningitis-arteritis (SRMA) occurs as an immune-mediated, inflammatory, and non-infectious disorder of juvenile and young-adult dogs. In principle,... (Review)
Review
Steroid-responsive meningitis-arteritis (SRMA) occurs as an immune-mediated, inflammatory, and non-infectious disorder of juvenile and young-adult dogs. In principle, SRMA is divided into two clinical courses: during the typical acute form, dogs are presented with fever, cervical hyperaesthesia, and reluctance to move. The more protracted form most probably emerges after insufficient immunosuppressive treatment or relapses, with additional neurologic deficits localized in the cervical and thoracolumbar spinal cord or multifocally. The trigger leading to SRMA still remains an unsolved riddle for immunologists and clinical neurologists. In the past, many attempts have been made to clarify the etiology of this disease without success. The purpose of writing this narrative review about SRMA is to summarize new insights on the pathogenesis of SRMA with a focus on immunologic dysregulation. Furthermore, unusual manifestations of the disease, new diagnostic approaches using possible laboratory biomarkers or diagnostic imaging tools, and potential innovative treatment strategies are discussed.
Topics: Animals; Dogs; Meningitis; Arteritis; Biomarkers; Steroids; Dog Diseases
PubMed: 37704169
DOI: 10.1016/j.tvjl.2023.106030