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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 -
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 -
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 -
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 -
La Tunisie Medicale May 2023Takayasu's Arteritis (TA) is a systemic vasculitis affecting the aorta and its main branches.
INTRODUCTION
Takayasu's Arteritis (TA) is a systemic vasculitis affecting the aorta and its main branches.
AIM
To describe the epidemiological, diagnostic, therapeutic and prognostic profile of TA in the referral departments of internal medicine in the Sousse region (Tunisia).
METHODS
This is a descriptive, retrospective and exhaustive study, carried out in the two departments of Internal Medicine of Sousse. Patients followed for AT, from 1996 to 2020 were included. The disease was defined according to the classification criteria of the American College of Rheumatology. Disease activity was assessed according to NIH criteria. Age referred to the date of diagnosis.
RESULTS
The study population consisted of 40 patients (Sahloul: n=32, Hached: n=8) with a sex ratio=0.17 and a median age=35 years (IIQ=[30-41]). The median diagnostic delay was 5 months (IIQ=[2-14]). The main clinical sign was pulse abolition and/or decrease (78%). Aortic stenosis was the main arterial lesion found (98%). Treatment was based on corticosteroids (95%) and immunosuppressants (42%). The prognosis of TA was often active (62%), with vascular co-morbidity (60%) and iatrogenic complications (35%).
CONCLUSION
The epidemiological-clinical profile of AT in the region of Sousse (Tunisia) was characterized by a female predominance, a diagnostic delay, a clinical polymorphism, and evolution towards vascular co-morbidities.
Topics: Humans; Female; Adult; Male; Takayasu Arteritis; Retrospective Studies; Tunisia; Delayed Diagnosis; Prognosis
PubMed: 38372522
DOI: No ID Found -
International Heart Journal 2023Takayasu arteritis (TA or TAK) is a chronic large vessel vasculitis with predilection to affect the aorta and its branches. The new 2022 ACR/EULAR classification...
Takayasu arteritis (TA or TAK) is a chronic large vessel vasculitis with predilection to affect the aorta and its branches. The new 2022 ACR/EULAR classification criteria for Takayasu arteritis incorporated imaging characteristics as an absolute requirement. ESR and CRP fails in accuracy as disease activity markers. Pentraxin 3 appears to be a relatively superior biomarker, which correlates with ITAS 2010 as per several studies. PET-CT is also increasingly being studied for assessing disease activity with variable results. The management of TAK involves use of steroids with upfront steroid sparing immunosuppressive agents. MMF is one such conventional DMARD/immunosuppressant with good efficacy and better safety profile, as reported in various cohort studies. Tocilizumab is proved to be a rapid remission inducing agent in refractory Takayasu arteritis in observational studies. TNF inhibitors in many uncontrolled studies showed good responses, and there is a need for good RCTs for confirmation. JAK inhibitors have also been used with success in a few reports.
Topics: Humans; Treatment Outcome; Takayasu Arteritis; Positron Emission Tomography Computed Tomography; Immunosuppressive Agents; Steroids
PubMed: 37518335
DOI: 10.1536/ihj.23-195