Giant cell arteritis

Giant cell arteritis is a medium-vessel vasculitis that affects both men and women. Because the disease commonly presents with nonspecific complaints stemming from cranial arterial insufficiency, the challenge for the physician is recognizing the diagnosis. Recognition of the entity and expeditious initiation of therapy are required to prevent permanent complications, including blindness. There is no pathognomonic finding on physical examination, blood testing, or commonly used radiologic investigations to confirm the diagnosis or establish disease activity. Oral corticosteroids are the mainstay of therapy. Other immune system modulators have no demonstrated efficacy and require further investigation. Percutaneous or surgical revascularization is a viable therapeutic option when the disease is not active.     

Giant cell arteritis

Giant cell arteritis (GCA) is the most common vasculitis of the elderly. The diagnosis can be challenging at times because of the limitation of the American Rheumatology Association (ARA) classification criteria and the significant proportion of biopsy-negative patients with GCA. We discuss the role of advanced imaging techniques, including positron emission tomography (PET) scanning, in establishing diagnosis and improved histopathology techniques to improve the sensitivity of temporal artery biopsy.There have been significant advances in the understanding of the pathogenesis of GCA, particularly the role of cytokine pathways such as the interleukins, IL-6-IL-17 axis, and the IL-12-interferon-γ axis and their implication for new therapies. We highlight that glucocorticoids remain the primary treatment for GCA, but recognize the risk of steroid-induced side effects. A number of pharmacotherapies to enable glucocorticoid dose reduction and prevent relapse have been studied.Early diagnosis and fast-track pathways have improved outcomes by encouraging adherence to evidence-based practice.     

Giant cell arteritis

Giant cell arteritis (GCA) is an immune-mediated chronic vasculitis of large- and medium-sized vessels usually occurring in White individuals aged over 50 years, in Western countries. The pathological hallmark of GCA is granulomatous inflammation of the involved vessels. Headache of new-onset is the most common clinical manifestation, and permanent vision loss is the most feared complication of GCA. The level of clinical suspicion for GCA should be based upon patient age, clinical symptoms, laboratory evaluation, and imaging findings. However, the diagnostic gold standard is achieved by histologic confirmation by temporal artery biopsy. Corticosteroids remain the only proven treatment for GCA and the prognosis for visual recovery is poor.     

Giant cell arteritis

Giant cell arteritis (GCA) or temporal arteritis is the most frequent systemic vasculitis in our area, and its incidence may be on the increase in the last years. GCA involves large and medium-sized vessels, with preference to the extracranial arteries, and it affects persons older than 50 years. The aetiopathogenesis is unknown, although several infectious agents may be implicated. A normal temporal artery biopsy do not exclude a GCA since the lesions may be skip. The most feared complications of GCA are permanent visual loss, ischemic strokes and thoracic and abdominal aortic aneurysms. The treatment consists of high-dose corticoids, and nowadays there is no effective therapeutic alternative without important adverse effects. The mortality of treated patients with GCA does not seem to be increased, probably due to a correct diagnosis and management of this entity.     

Giant cell arteritis.

Giant cell arteritis is a generalized inflammatory disorder involving large and medium-sized arteries. The etiology is unknown, although an autoimmune pathogenesis seems probable. In view of the clinical similarities between patients with positive biopsy findings for polymyalgia rheumatica and those with negative biopsy findings, many authors favor the concept that polymyalgia rheumatica is an expression of an underlying giant cell arteritis. There is, however, still controversy as to whether polymyalgia rheumatica and temporal arteritis are different expressions of one and the same disease or two separate, partly overlapping types of giant cell arteritis. A single etiologic factor may be responsible for the two conditions, sometimes expressing itself as polymyalgia rheumatica and sometimes as giant cell arteritis. Recent findings of morphologic similarities in terms of arterial wall atrophy, calcifications, and inflammatory reactions may indicate that polymyalgia rheumatica and temporal arteritis represent different degrees or stages of the same disease.     

Giant cell arteritis.

Giant cell arteritis (GCA), the most common form of systemic vasculitis in adults, preferentially involves large and medium-sized arteries in patients over the age of 50. The classic manifestations are headache, jaw claudication, polymyalgia rheumatica (PMR), and visual symptoms, but 40% of patients present with a wide range of occult manifestations. Early diagnosis and treatment with prednisone can prevent blindness, the most feared complication of GCA. The pathogenesis of GCA is T-cell dependent and antigen driven. Clinical subsets of GCA appear to result from variable cytokine expression. The risk of developing thoracic aortic aneurysm is increased more than 17-fold in patients with GCA. GCA can also involve large arteries, especially the subclavian and axillary arteries. Color Doppler ultrasound, magnetic resonance imaging, and positron-emission tomography scanning are providing insights into the extent and pathogenesis of the disease but have not replaced temporal artery biopsy as the gold standard for securing the diagnosis. Two recently completed double-blind, placebo-controlled trials concerning whether methotrexate plus prednisone is more effective than prednisone alone reached conflicting conclusions.     

Giant right ventricular mural vegetation mimicking a cardiac tumour

Mural vegetations in the course of infective endocarditis are very rare. We report the case of a patient with an extremely large right ventricular free wall vegetation. Establishing diagnosis in the presence of only mural vegetations on echocardiography scan without valve involvement in the inflammatory process was difficult. In a differential diagnosis, benign and malignant tumours, metastases and thrombi were taken into account. The patient was operated upon and the tumour was removed successfully. A histopathological examination revealed an inflammatory character of the tumour. The patient was treated according to antibiogram and discharged home in stable condition.     

Giant cell and Takayasu arteritis

PURPOSE OF REVIEW: Giant cell arteritis and Takayasu arteritis are well known large vessel vasculitides with an unknown etiology. As they have similar clinical features, this short article reviews recent advances in clinical and pathophysiological findings, focusing in particular on papers published in the past year. RECENT FINDINGS: Delayed gadolinium-enhanced magnetic resonance imaging showed delayed hyperenhancement in the aortic wall in Takayasu arteritis. This technique may be useful in monitoring disease activity or inflammation in the arterial wall and can be used for small vessels such as temporal arteries in giant cell arteritis with high-resolution imaging. Evidence is accumulating that antitumor necrosis factor-alpha monoclonal antibody therapy can be useful for patients refractory to corticosteroid and/or immunosuppressant treatment. Functional promoter polymorphisms of genes encoding inducible nitric oxide synthase and I-kappaB-like protein were suggested to be associated with susceptibility to giant cell arteritis and Takayasu arteritis, respectively. SUMMARY: Advances in imaging technique will make it possible to evaluate inflammatory activity of the vascular lesions and provide a useful guide for treatment of giant cell arteritis and Takayasu arteritis. Further understanding of the pathophysiological mechanism may contribute to the development of new medicine targeting critical factors in the pathogenesis, such as antitumor necrosis factor-alpha agents.     

Giant cell arteritis: a systemic vascular disease

Giant cell arteritis (GCA) is increasingly being recognized as a systemic vascular disease, not confined to the cranial arteries. Epidemiological studies have shown that almost one-third of the patients with GCA develop serious peripheral vascular complications during long-term follow up, and there is growing evidence that unrecognized extracranial involvement may be even more common. GCA of large- and medium-sized peripheral arteries typically leads to long tapering and occlusion of the arterial lumen due to concentric intimal thickening, sometimes accompanied by spontaneous dissection. Depending on the extent of the arterial obliteration and on the anatomy of the involved arterial segment, this may result in severe ischemia of the limbs during the acute phase of the disease. GCA of the aorta usually remains asymptomatic for many years, and leads to a markedly increased risk of aneurysms and dissections, particularly of the thoracic aorta. Evolving vascular imaging techniques such as duplex ultrasound, computer tomography (CT), magnetic resonance imaging (MRI), and fluorine-18-desoxyglucose positron emission tomography (18F-FDG-PET) have greatly improved our ability to detect and study arterial changes in large-artery vasculitis. Boosted by these advances in vascular imaging, vascular specialists are increasingly involved in the early diagnosis, follow-up and treatment of patients with large-vessel vasculitis.