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Zeitschrift Fur Rheumatologie Aug 2020Large vessel vasculitides comprise two distinct entities, giant cell arteritis (GCA) and Takayasu arteritis (TAK). GCA is the most common vasculitis in central Europe,... (Review)
Review
Large vessel vasculitides comprise two distinct entities, giant cell arteritis (GCA) and Takayasu arteritis (TAK). GCA is the most common vasculitis in central Europe, becoming manifested at an age over 50 years. In contrast, the much rarer TAK affects almost exclusively young adults and mostly women. Both vasculitides are granulomatous arteritides affecting mainly the aorta and its major arterial branches. GCA and TAK are associated with different major histocompatibility complex genes. Infections possibly play a role in the initiation of large vessel vasculitides. Activation of dendritic cells in the adventitia induces chemokine and cytokine-mediated recruitment and maturation of T‑helper (Th)1 and Th17 cells and macrophages producing cytokines, growth factors and matrix metalloproteinases. In GCA, CD4+ T‑helper cells and macrophages are predominantly found in the inflammatory infiltrate. In TAK, the infiltrate also contains cytotoxic CD8+ T‑cells and γδ T‑cells. This could indicate different antigenic triggers in GCA and TAK. Inflammatory infiltration with T‑cells and macrophages and activation of myofibroblasts and smooth muscular cells induce vascular remodeling with intimal hyperplasia and destruction of the media. Remodeling is histologically characterized by progressive arterial wall fibrosis, vascular stenosis and obstruction. In summary, GCA and TAK represent two different entities with a distinct human leukocyte antigen (HLA) and potentially etiopathogenetic background. Clinically, inflammation-related general symptoms and signs of ischemia are encountered, accompanied by increased levels of serological markers of inflammation.
Topics: Adult; Cytokines; Europe; Female; Giant Cell Arteritis; Humans; Macrophages; Male; Takayasu Arteritis; Young Adult
PubMed: 32430566
DOI: 10.1007/s00393-020-00809-z -
Zeitschrift Fur Rheumatologie Feb 2022Primary systemic vasculitides can be observed at any age. Some vasculitides occur preferentially in childhood, such as Kawasaki syndrome or immunoglobulin A (IgA)... (Review)
Review
Primary systemic vasculitides can be observed at any age. Some vasculitides occur preferentially in childhood, such as Kawasaki syndrome or immunoglobulin A (IgA) vasculitis, whereas others, such as giant cell arteritis, occur beyond the age of 50 years. Vasculitides occurring in childhood or adolescence and adulthood may have different phenotypes, different disease courses and outcomes depending on the age of manifestation. For example, those with Takayasu arteritis beginning in adolescence have different vascular involvement, a higher degree of systemic inflammation and a more aggressive course of disease than those with adult-onset disease. In contrast, IgA vasculitis is more severe in adults than in children. The causes for the age predilections and different age-dependent disease manifestations have not yet been clarified. The therapeutic principles are similar for vasculitides occurring in children or adolescents and adults. The first international evidence-based treatment recommendations are now available for juvenile vasculitides, although the evidence for certain forms of treatment is still very limited. The treatment of adult vasculitides can be guided by numerous national and international guidelines and recommendations. Many vasculitides carry a high risk of morbidity and mortality and the timely detection and treatment are therefore necessary. In this article, similarities and differences in the clinical presentations, treatment, courses and prognosis of vasculitides in children or adolescents and adults are discussed.
Topics: Adolescent; Adult; Disease Progression; Giant Cell Arteritis; Granulomatosis with Polyangiitis; Humans; IgA Vasculitis; Middle Aged; Polyarteritis Nodosa; Prognosis; Takayasu Arteritis
PubMed: 34978582
DOI: 10.1007/s00393-021-01141-w -
Headache Sep 2014Giant cell arteritis (GCA) is a medium and large-vessel vasculitis, which is an important cause of secondary headache in older adults. While GCA has a classic... (Review)
Review
Giant cell arteritis (GCA) is a medium and large-vessel vasculitis, which is an important cause of secondary headache in older adults. While GCA has a classic presentation occurring after the age of 50, atypical presentations (eg, fever of unknown origin, cough, low or normal erythrocyte sedimentation rate) may lead to a delay in diagnosis. The topography of vascular involvement has implications for disease-related complications, which can result in neurologic disease at multiple levels of the nervous system. The most feared complication, vision loss, fortunately becomes uncommon after initiation of corticosteroids. Corticosteroid treatment should not be withheld while waiting the results of a temporal artery biopsy (TAB), which remains the gold standard for GCA diagnosis. Newer diagnostic modalities, including ultrasound, magnetic resonance imaging, and positron emission tomography can play an important role in directing treatment in cases with negative TAB. After successful control of the disorder, patients should be gradually tapered off corticosteroids, with careful monitoring using both clinical and laboratory parameters to assess for relapse. Corticosteroid-related treatment complications are not uncommon in GCA. There is mixed evidence for use of adjunct corticosteroid-sparing agents (eg, methotrexate), although these should be initiated in the setting of corticosteroid-related morbidity and/or cases with frequent relapse.
Topics: Giant Cell Arteritis; Humans
PubMed: 25041449
DOI: 10.1111/head.12425 -
Asia-Pacific Journal of Ophthalmology...Giant cell arteritis and Takayasu arteritis are large-vessel vasculitides that share multiple common features but also have significant differences in epidemiology,... (Review)
Review
Giant cell arteritis and Takayasu arteritis are large-vessel vasculitides that share multiple common features but also have significant differences in epidemiology, demographics, clinical presentation, evaluation, and treatment. Giant cell arteritis is more common in elderly patients of Caucasian descent versus Takayasu arteritis, which is more prevalent in younger patients of Asian descent. Although traditionally age has been the main criterion for differentiating the 2 etiologies, modifications in the diagnostic criteria have recognized the overlap between the 2 conditions. In this monograph, we review the diagnostic criteria for both conditions and describe the epidemiology, pathogenesis, histology, evaluation, and management for large-vessel vasculitis in ophthalmology. Additionally, we describe ocular imaging techniques that may be utilized by ophthalmologists to identify manifestations of large-vessel vasculiti- des in patients. Lastly, we compare and contrast the key clinical, laboratory, and pathologic features that might help ophthalmologists to differentiate the 2 entities.
Topics: Aged; Giant Cell Arteritis; Humans; Ophthalmology; Takayasu Arteritis
PubMed: 35533336
DOI: 10.1097/APO.0000000000000514 -
Journal of Nephrology Aug 2016Medium- and large-vessel vasculitides (MVV and LVV, respectively) comprise a heterogeneous group of disorders whose common denominator is the inflammatory involvement of... (Review)
Review
Medium- and large-vessel vasculitides (MVV and LVV, respectively) comprise a heterogeneous group of disorders whose common denominator is the inflammatory involvement of vessels of medium and large size. This disease spectrum includes giant-cell arteritis and Takayasu's arteritis, which typically affect the aorta and its main branches, and Kawasaki's disease and polyarteritis nodosa, which involve medium-sized arteries. Chronic periaortitis, characterized by a perivascular fibro-inflammatory reaction affecting the abdominal aorta and the periaortic tissue, frequently has a systemic distribution, involving other segments of the aorta and its major branches, and could thus be included in this group. Unlike small-vessel vasculitides, MVV and LVV do not cause glomerulonephritis, although glomerular immune-mediated lesions and tubulo-interstitial nephritis occur with varying frequency. However, MVV and LVV can often involve the renal artery and its branches, causing a wide array of lesions that range from renal artery stenosis to intra-renal vasculitis causing renal ischaemia/infarction, microaneurysms and haemorrhage. This review focuses on renal involvement in MVV and LVV and underlines why renal abnormalities in these syndromes should not be overlooked.
Topics: Giant Cell Arteritis; Humans; Kidney Diseases; Mucocutaneous Lymph Node Syndrome; Polyarteritis Nodosa; Retroperitoneal Fibrosis; Takayasu Arteritis
PubMed: 27098921
DOI: 10.1007/s40620-016-0303-8 -
Best Practice & Research. Clinical... Feb 2018The prognosis of ANCA-associated vasculitis has been transformed in recent years. Once it was a set of invariably acute and fatal conditions, but these disorders are... (Review)
Review
The prognosis of ANCA-associated vasculitis has been transformed in recent years. Once it was a set of invariably acute and fatal conditions, but these disorders are currently considered to be chronic diseases. This change is largely attributable to earlier diagnosis and the careful application of immunotherapeutics. However, patients still experience premature mortality, relapse, comorbid ill health and poor quality of life. Mortality rates in large-vessel vasculitis are not comparable; however, morbidity and poor patient outcomes prevail. Toxicity secondary to glucocorticoids represents a common driver of poor outcomes across systemic vasculitis. The main thrust of future treatment strategies is to reduce if not eliminate exposure to these agents.
Topics: Giant Cell Arteritis; Humans; Prognosis; Quality of Life; Systemic Vasculitis; Takayasu Arteritis
PubMed: 30526894
DOI: 10.1016/j.berh.2018.08.011 -
Neurology Oct 2017
Topics: Adult; Arteritis; Carotid Artery Diseases; Female; Humans; Magnetic Resonance Imaging; Neck Pain; Syndrome; Ultrasonography, Doppler, Color
PubMed: 28993524
DOI: 10.1212/WNL.0000000000004502 -
International Journal of Rheumatic... Jan 2019We discuss recent and prospective research in small and large vessel vasculitis. Large cohorts of Takayasu arteritis (TA) have been recently published from across the... (Review)
Review
We discuss recent and prospective research in small and large vessel vasculitis. Large cohorts of Takayasu arteritis (TA) have been recently published from across the world, clarifying our understanding of this uncommon disease. Novel open-ended approaches like large-scale genotyping, proteomics and metabolomics have helped gain novel insights into TA, giant cell arteritis (GCA) and anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV). Recent advances in the imaging of TA and GCA offer promise for earlier diagnosis and better monitoring of response to therapy. Although two randomized controlled trials of abatacept and tocilizumab failed to meet their primary end-points, successful large-scale studies of abatacept and tocilizumab in GCA hold promise for better disease control. While cyclophosphamide has revolutionized the management of AAV, increasing use of rituximab as an alternative induction regimen, as well as use of novel approaches involving reduced or no corticosteroid use for AAV and alternative agents such as avacopan (a complement 5a receptor antagonist) hold promise for lesser toxic induction regimens in the future. Increasingly, the risk of cardiovascular events and comorbidities such as osteoporosis are being recognized as factors affecting long-term prospects of patients with vasculitis. There is a shift in emphasis to utilize patient-reported outcomes to more accurately gauge the impact of vasculitides and their treatment.
Topics: Animals; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis; Biomedical Research; Diffusion of Innovation; Forecasting; Giant Cell Arteritis; Humans; Immunosuppressive Agents; Risk Factors; Takayasu Arteritis; Treatment Outcome
PubMed: 30168260
DOI: 10.1111/1756-185X.13370 -
Toxicologic Pathology Feb 2018Arteritis/polyarteritis occurs spontaneously in many species used in preclinical toxicology studies. In Göttingen minipigs, arteritis/polyarteritis is an occasionally... (Review)
Review
Arteritis/polyarteritis occurs spontaneously in many species used in preclinical toxicology studies. In Göttingen minipigs, arteritis/polyarteritis is an occasionally observed background change. In the minipig, this finding differs in frequency and nature from age-related polyarteritis nodosa in rats or monkeys, and Beagle pain syndrome in dogs. In minipigs, it can be present in a single small- or medium-sized artery of an organ or a few organs and is most commonly recorded in the cardiac and extracardiac blood vessels, vagina, oviduct, rectum, epididymis, spinal cord, pancreas, urinary bladder, kidneys, and stomach. The etiology is unknown although it has been considered in minipigs as well as in rats, dogs, and monkeys to be possibly immune mediated. This background change is important with respect to its nature and distribution in the minipig in order to distinguish it from drug-induced vascular changes, which might occur in similar locations and have similar morphologic features. This review summarizes the morphology, incidence, and predilection sites of arteritis as a spontaneously occurring background change and as a drug-induced vasculopathy in the minipig, and also describes the main aspects to consider when evaluating vascular changes in Göttingen minipig toxicity studies and their human relevance.
Topics: Animals; Arteritis; Disease Models, Animal; Polyarteritis Nodosa; Swine; Swine, Miniature
PubMed: 29471777
DOI: 10.1177/0192623318754791 -
QJM : Monthly Journal of the... Dec 2020
Topics: Giant Cell Arteritis; Humans
PubMed: 32176301
DOI: 10.1093/qjmed/hcaa095