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Frontiers in Immunology 2021Giant cell arteritis (GCA) is a granulomatous systemic vasculitis of large- and medium-sized arteries that affects the elderly. In recent years, advances in diagnostic... (Review)
Review
Giant cell arteritis (GCA) is a granulomatous systemic vasculitis of large- and medium-sized arteries that affects the elderly. In recent years, advances in diagnostic imaging have revealed a greater degree of large vessel involvement than previously recognized, distinguishing classical cranial- from large vessel (LV)- GCA. GCA often co-occurs with the poorly understood inflammatory arthritis/bursitis condition polymyalgia rheumatica (PMR) and has overlapping features with other non-infectious granulomatous vasculitides that affect the aorta, namely Takayasu Arteritis (TAK) and the more recently described clinically isolated aortitis (CIA). Here, we review the literature focused on the immunopathology of GCA on the background of the three settings in which comparisons are informative: LV and cranial variants of GCA; PMR and GCA; the three granulomatous vasculitides (GCA, TAK, and CIA). We discuss overlapping and unique features between these conditions across clinical presentation, epidemiology, imaging, and conventional histology. We propose a model of GCA where abnormally activated circulating cells, especially monocytes and CD4 T cells, enter arteries after an unknown stimulus and cooperate to destroy it and review the evidence for how this mechanistically occurs in active disease and improves with treatment.
Topics: Animals; Aorta; CD4-Positive T-Lymphocytes; Giant Cell Arteritis; Humans; Inflammation Mediators; Monocytes; Takayasu Arteritis; Temporal Arteries
PubMed: 33717128
DOI: 10.3389/fimmu.2021.623716 -
Archives of Pathology & Laboratory... Jul 2014Eosinophilic coronary monoarteritis is an unfamiliar cause of acute myocardial ischemia. Most commonly, it presents as a left-sided chest pain or sudden death in... (Review)
Review
Eosinophilic coronary monoarteritis is an unfamiliar cause of acute myocardial ischemia. Most commonly, it presents as a left-sided chest pain or sudden death in middle-aged women with no traditional risk factors for coronary artery disease. Because the abrupt onset leaves almost no time for intervention, the symptoms readily lead to death, and most cases are diagnosed at necropsy. Dissection of the coronary artery wall with resultant occlusion of the lumen, which commonly affects the left anterior descending artery, is a consistent gross finding. An inflammatory infiltrate, which is predominantly composed of eosinophils in the tunica adventitia and tunica media and is often accompanied by a hematoma in between these 2 layers, is observed histologically. The etiology remains unclear, but an increase in the activity of eosinophils because of hormonal interactions during pregnancy has been suggested. Interplay of hormones is thought to culminate in the release of histolytic agents by the eosinophils, which initiate the dissection process. Currently, there is no specific treatment for eosinophilic coronary monoarteritis, but cyclophosphamide and prednisone have shown positive results in the treatment of spontaneous coronary artery dissection with unspecified periadventitial inflammation. Percutaneous coronary procedures have also resulted in favorable outcomes in a subset of patients. Because of the high, sudden death rate in eosinophilic coronary monoarteritis, deciphering the underlying pathophysiology of this almost invariably fatal disease remains both a challenge and a key to developing screening methods that will allow timely detection and thus treatment.
Topics: Adult; Aortic Dissection; Arteritis; Coronary Aneurysm; Coronary Artery Disease; Coronary Vessels; Death, Sudden, Cardiac; Diagnosis, Differential; Eosinophilia; Female; Humans; Male; Middle Aged; Pregnancy; Puerperal Disorders; Risk Factors
PubMed: 24978927
DOI: 10.5858/arpa.2012-0610-RS -
JNMA; Journal of the Nepal Medical... Dec 2022Takayasu's arteritis is a chronic vasculitis of medium and large vessels. The most involved vessel is the aorta and its major branches. The disease is primarily seen in...
UNLABELLED
Takayasu's arteritis is a chronic vasculitis of medium and large vessels. The most involved vessel is the aorta and its major branches. The disease is primarily seen in young women. The described incidence of the disease ranges from 0.3 to 3.3 million per year. The vessels are characterized by mononuclear infiltration and granulomatous inflammation of vascular media, which leads to arterial wall thickening with stenosis, occlusion, and aneurysmal dilation. Here we present a case of Takayasu's arteritis in a 26-year-old woman who presented with syncope and dizziness with thickened walls of the arch of the aorta and its branches in Magnetic Resonance Imaging angiogram finding. Women of Japanese descent are not the only ones who can develop Takayasu's arteritis; it can affect anyone. Therefore, early diagnosis and treatment are warranted. When the disease is dormant, the outcome seems favourable.
KEYWORDS
aortitis syndrome; arteritis; case reports; pulseless disease; young female arteritis.
Topics: Female; Humans; Adult; Takayasu Arteritis; Aorta; Magnetic Resonance Imaging
PubMed: 36705112
DOI: 10.31729/jnma.7685 -
The New England Journal of Medicine Jul 2014A 79-year-old woman presents with new-onset pain in her neck and both shoulders. She takes 7.5 mg of prednisone per day for giant-cell arteritis. Occipital tenderness... (Review)
Review
A 79-year-old woman presents with new-onset pain in her neck and both shoulders. She takes 7.5 mg of prednisone per day for giant-cell arteritis. Occipital tenderness and diplopia developed 11 months before presentation. At that time, her erythrocyte sedimentation rate was elevated, at 78 mm per hour, and a temporal-artery biopsy revealed granulomatous arteritis. The diplopia resolved after 6 days of treatment with 60 mg of prednisone daily. Neither headache nor visual symptoms developed when the glucocorticoids were tapered. How should this patient’s care be managed?
Topics: Aged; Diplopia; Female; Giant Cell Arteritis; Humans; Immunosuppressive Agents; Polymyalgia Rheumatica
PubMed: 24988557
DOI: 10.1056/NEJMcp1214825 -
Internal Medicine (Tokyo, Japan) 2023A 74-year-old woman was admitted because of malaise and a low-grade fever. Her C-reactive protein level was 0.96 mg/dL. Computed tomography (CT) revealed diffuse uniform...
A 74-year-old woman was admitted because of malaise and a low-grade fever. Her C-reactive protein level was 0.96 mg/dL. Computed tomography (CT) revealed diffuse uniform thickening of the arterial wall from the abdominal aorta to the common iliac artery and right hydronephrosis. F-fluordesoxyglucose positron emission tomography-CT showed an accumulation in the same area. These findings suggested Takayasu arteritis and retroperitoneal fibrosis as differential diagnoses. Takayasu arteritis is characterized by thickening of the arterial walls, and retroperitoneal fibrosis is characterized by membranous lesions covering the outer surface of the arterial walls. Thus, Takayasu arteritis was deemed the most likely diagnosis. Steroid treatment was effective.
Topics: Female; Humans; Aged; Takayasu Arteritis; Retroperitoneal Fibrosis; Tomography, X-Ray Computed; Aorta, Abdominal; Arteries
PubMed: 37258208
DOI: 10.2169/internalmedicine.0305-22 -
Clinical and Experimental Rheumatology Apr 2023
Topics: Humans; Polymyalgia Rheumatica; Giant Cell Arteritis; Fluorodeoxyglucose F18
PubMed: 36995322
DOI: 10.55563/clinexprheumatol/3bozph -
RMD Open Jun 2023In this large multicentre study, we compared the effectiveness and safety of tocilizumab intravenous versus subcutaneous (SC) in 109 Takayasu arteritis (TAK) patients.
OBJECTIVES
In this large multicentre study, we compared the effectiveness and safety of tocilizumab intravenous versus subcutaneous (SC) in 109 Takayasu arteritis (TAK) patients.
METHODS
We conducted a retrospective multicentre study in referral centres from France, Italy, Spain, Armenia, Israel, Japan, Tunisia and Russia regarding biological-targeted therapies in TAK, since January 2017 to September 2019.
RESULTS
A total of 109 TAK patients received at least 3 months tocilizumab therapy and were included in this study. Among them, 91 and 18 patients received intravenous and SC tocilizumab, respectively. A complete response (NIH <2 with less than 7.5 mg/day of prednisone) at 6 months was evidenced in 69% of TAK patients, of whom 57 (70%) and 11 (69%) patients were on intravenous and SC tocilizumab, respectively (p=0.95). The factors associated with complete response to tocilizumab at 6 months in multivariate analysis, only age <30 years (OR 2.85, 95% CI 1.14 to 7.12; p=0.027) and time between TAK diagnosis and tocilizumab initiation (OR 1.18, 95% CI 1.02 to 1.36; p=0.034). During the median follow-up of 30.1 months (0.4; 105.8) and 10.8 (0.1; 46.4) (p<0.0001) in patients who received tocilizumab in intravenous and SC forms, respectively, the risk of relapse was significantly higher in TAK patients on SC tocilizumab (HR=2.55, 95% CI 1.08 to 6.02; p=0.033). The overall cumulative incidence of relapse at 12 months in TAK patients was at 13.7% (95% CI 7.6% to 21.5%), with 10.3% (95% CI 4.8% to 18.4%) for those on intravenous tocilizumab vs 30.9% (95% CI 10.5% to 54.2%) for patients receiving SC tocilizumab. Adverse events occurred in 14 (15%) patients on intravenous route and in 2 (11%) on SC tocilizumab.
CONCLUSION
In this study, we confirm that tocilizumab is effective in TAK, with complete remission being achieving by 70% of disease-modifying antirheumatic drugs-refractory TAK patients at 6 months.
Topics: Humans; Adult; Retrospective Studies; Takayasu Arteritis; Treatment Outcome; Antirheumatic Agents
PubMed: 37321669
DOI: 10.1136/rmdopen-2022-002830 -
European Journal of Vascular and... Mar 2015
Topics: Adrenal Cortex Hormones; Adult; Blood Vessel Prosthesis Implantation; Carotid Arteries; Female; Humans; Magnetic Resonance Angiography; Multimodal Imaging; Predictive Value of Tests; Takayasu Arteritis
PubMed: 25308759
DOI: 10.1016/j.ejvs.2014.09.003 -
BMC Cardiovascular Disorders Jan 2021Cardiac vasculitis is recognized as a heterogeneous disease process with a wide spectrum of manifestations including pericarditis, myocarditis, valvular heart disease... (Review)
Review
Cardiac vasculitis is recognized as a heterogeneous disease process with a wide spectrum of manifestations including pericarditis, myocarditis, valvular heart disease and less frequently, coronary artery vasculitis (CAV). CAV encompasses an emerging field of diseases which differ from conventional atherosclerotic disease and have a proclivity for the younger population groups. CAV portends multiple complications including the development of coronary artery aneurysms, coronary stenotic lesions, and thrombosis, all which may result in acute coronary syndromes. There are several aetiologies for CAV; with Kawasaki's disease, Takayasu's arteritis, Polyarteritis Nodosa, and Giant-Cell Arteritis more frequently described clinically, and in literature. There is a growing role for multi-modality imaging in assisting the diagnostic process; including transthoracic echocardiography, cardiac magnetic resonance imaging, computed tomography coronary angiography, fluorodeoxyglucose-positron emission tomography and conventional coronary angiogram with intravascular ultrasound. Whilst the treatment paradigms fundamentally vary between different aetiologies, there are overlaps with pharmacological regimes in immunosuppressive agents and anti-platelet therapies. Interventional and surgical management are is a consideration in select populations groups, within a multi-disciplinary context. Further large-scale studies are required to better appropriately outline management protocols in this niche population.
Topics: Cardiac Imaging Techniques; Coronary Artery Disease; Giant Cell Arteritis; Humans; Mucocutaneous Lymph Node Syndrome; Multimodal Imaging; Polyarteritis Nodosa; Predictive Value of Tests; Prognosis; Takayasu Arteritis
PubMed: 33407141
DOI: 10.1186/s12872-020-01813-6 -
Best Practice & Research. Clinical... Feb 2018Vascular surgery remains an important option in the management of Takayasu arteritis (TA). Its use is predominantly confined to the treatment of symptomatic organ... (Review)
Review
Vascular surgery remains an important option in the management of Takayasu arteritis (TA). Its use is predominantly confined to the treatment of symptomatic organ ischaemia or life-threatening aneurysm formation. In most cases, this follows the failure of medical therapy to prevent arterial injury. Open surgery and endovascular approaches are used. The choice between them, at least in part, is determined by the site and nature of the lesion. Open surgery, although more invasive, offers enhanced duration of arterial patency, whereas for endovascular intervention, primary angioplasty without stenting is preferred, with stenting reserved for primary or secondary angioplasty failures. Although there is increasing interest in the role of stent grafts and tailor-made endovascular stents, long-term outcomes remain to be reported. Interventional outcomes are improved and complications reduced by therapeutic control of disease activity before and after surgery. The wider use of combined immunosuppression and the introduction of biologic therapy for refractory TA may reduce future requirements for surgical intervention.
Topics: Angioplasty; Humans; Takayasu Arteritis; Treatment Outcome
PubMed: 30526891
DOI: 10.1016/j.berh.2018.07.008