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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 -
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 -
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 -
Clinical & Experimental Optometry Sep 2020Temporal arteritis (TA), or giant cell arteritis, is a systemic autoimmune vasculitis affecting patients over 50 years of age. It can cause rapid, irreversible... (Review)
Review
Temporal arteritis (TA), or giant cell arteritis, is a systemic autoimmune vasculitis affecting patients over 50 years of age. It can cause rapid, irreversible bilateral vision loss in older adults and is therefore considered an ophthalmological emergency. Many of the symptoms and signs of TA can be vague, non-specific and gradual in onset, often leading to a delayed or inaccurate diagnosis. As such, it is important for a wide variety of primary optometrists and health practitioners to maintain a robust understanding of the clinical presentation, key investigations and time-sensitive management of this disease, as early initiation of treatment for TA can be vision- and life-saving.
Topics: Biopsy; Diagnosis, Differential; Disease Management; Giant Cell Arteritis; Humans; Positron Emission Tomography Computed Tomography; Temporal Arteries
PubMed: 31663193
DOI: 10.1111/cxo.12975 -
Eye (London, England) Jun 2020The landscape of the investigation and management of giant cell arteritis (GCA) is advancing. In this review we will outline the recent advances by searching the current... (Review)
Review
The landscape of the investigation and management of giant cell arteritis (GCA) is advancing. In this review we will outline the recent advances by searching the current English literature for relevant articles using key words of giant cell arteritis, temporal arteritis, Horton's disease, investigation, and treatment. Delay in diagnosis, diagnostic uncertainty and glucocorticoid (GC) morbidity are among the highest concerns of clinicians and patients in this disease area. The positive news is that fast track pathways, imaging techniques and new therapies are emerging for routine management of GCA. Future directions for intervention in the treatment paradigm will be discussed.
Topics: Giant Cell Arteritis; Glucocorticoids; Humans
PubMed: 31582795
DOI: 10.1038/s41433-019-0608-7 -
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 -
Best Practice & Research. Clinical... Mar 2023The recent publication of the American College of Rheumatology (ACR)-European Alliance of Associations for Rheumatology (EULAR) classification criteria for large vessel... (Review)
Review
The recent publication of the American College of Rheumatology (ACR)-European Alliance of Associations for Rheumatology (EULAR) classification criteria for large vessel vasculitis and anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAVs) has provided modern criteria for the classification of these conditions, which incorporate contemporary methods of investigation and diagnosis. Further validation is required in independent cohorts, especially from populations that were not well represented in the development cohort. Studies of the occurrence of large vessel vasculitis report that Takayasu arteritis is a rare disease in most populations, and giant cell arteritis is the most common vasculitis in older populations. The incidence of AAV appears to have plateaued, but the prevalence is increasing as a result of lower mortality. The new classification criteria may affect the reported incidence and prevalence, and studies will be needed to confirm this. The impact of COVID-19 on the occurrence of the vasculitides is not completely known, but there is evidence of reduced occurrence of Kawasaki disease and IgA-associated vasculitis following lockdowns with reduced transmission of possible trigger infectious agents.
Topics: Humans; Aged; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis; Giant Cell Arteritis; Takayasu Arteritis; Rheumatology
PubMed: 37468418
DOI: 10.1016/j.berh.2023.101845 -
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 -
Current Rheumatology Reports Jun 2020Vasculitides are characterized by mostly autoimmunologically induced inflammatory processes of vascular structures. They have various clinical and radiologic... (Review)
Review
PURPOSE OF REVIEW
Vasculitides are characterized by mostly autoimmunologically induced inflammatory processes of vascular structures. They have various clinical and radiologic appearances. Early diagnosis and reliable monitoring are indispensable for adequate therapy to prevent potentially serious complications. Imaging, in addition to laboratory tests and physical examination, constitutes a key component in assessing disease extent and activity. This review presents current standards and some typical findings in the context of imaging in vasculitis with particular attention to large vessel vasculitides.
RECENT FINDINGS
Recently, imaging has gained importance in the management of vasculitis, especially regarding large vessel vasculitides (LVV). Recently, EULAR (European League Against Rheumatism) has launched its recommendations concerning the diagnosis of LVVs. Imaging is recommended as the preferred complement to clinical examination. Color-coded duplex sonography is considered the first choice imaging test in suspected giant cell arteritis, and magnetic resonance imaging is considered the first choice in suspected Takayasu's arteritis. Due to diversity of clinical and radiologic presentations, diagnosis and therapy monitoring of vasculitides may constitute a challenge. As a result of ongoing technological progress, a variety of non-invasive imaging modalities now play an elemental role in the interdisciplinary management of vasculitic diseases.
Topics: Giant Cell Arteritis; Humans; Magnetic Resonance Imaging; Takayasu Arteritis; Vasculitis
PubMed: 32562073
DOI: 10.1007/s11926-020-00915-6