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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.

Antibodies, Antineutrophil Cytoplasmic↗

Small bowel infarction in association with giant cell arteritis.

Giant cell arteritis is not uncommonly found in extracranial arteries in postmortem studies of patients with temporal arteritis. Presentation with vasculitis involving extracranial arteries is, however, unusual. This report describes a case of giant cell arteritis presenting with and complicated by infarction of the small bowel. Following surgical resection of the infarcted segment of bowel and commencement of steroid therapy, the patient is now well and free of symptoms. The literature concerning extracranial and in particular small bowel giant cell arteritis is reviewed.

Aged↗

[A new approach towards giant cell arteritis].

Giant cell arteritis is a rare systemic vasculitis affecting large- and medium-sized arteries. Focal arteries lesions, include mononuclear cells infiltration of the vessel wall with giant cell formation. It is a disease of elderly persons and can result in a wide variety of systemic, neurological and ophthalmic complications, due to ischemia. The incidence of visual loss and ocular involvement varies between 14-88%, but one of the most common and severe complications is anterior ischemic optic neuropathy. The other ocular ischemic lesions include: central retinal artery occlusion, choroidal ischemia, diplopia, ocular motor paresis, anterior uveitis, cataract, ocular hypotony, corneal oedema and ulcerations, episcleritis and anterior scleritis, orbital cellulitis and pseudotumor. Because giant cell arteritis is potentially blinding disease, early diagnosis and immediate treatment with high dose corticosteroids may prevent further damage to the affected eye and prevent visual loss in the opposite eye. The purpose of this review is to revise established knowledge and to highlight the recent developments in diagnosis and management of giant cell arteritis.

Aged↗

Long-term outcome of giant cell arteritis.

Giant cell arteritis is usually a self-limiting disease with a variable duration of months to years. However, in a subset of patients the disease may follow a protracted course, requiring long-term treatment with glucocorticoids. To date, glucocorticoids are the only agents whose efficacy has been unquestionably proven. More specifically, they can both improve the clinical symptoms of giant cell arteritis and also prevent its complications, including visual loss. Glucocorticoids therapy is notoriously fraught with numerous side effects, therefore it is sensible to taper glucocorticoids as quickly as possible. Flares are not uncommon and tend often to occur upon tapering of glucocorticoids dosage or on withdrawal of glucocorticoids therapy. However, in most cases flares are mild and appear to respond favorably to an increase in glucocorticoids dosage or reintroduction of glucocorticoids therapy, respectively. Mortality rates of giant cell arteritis patients are comparable to those of the general population, but there is evidence for an increased frequency of potentially life-threatening ischemic events, such as myocardial infarction and cerebro-vascular accidents, especially early on in the disease course. The risk conferred by the disease appears to decrease with time, presumably as a consequence of glucocorticoids treatment, whereas it can remain significantly elevated in patients whose disease activity is not sufficiently controlled by the treatment. By contrast, there is no evidence that giant cell arteritis is associated with an increased prevalence of malignancies or that it may represent a paraneoplastic syndrome.

Blindness↗

[Pericardial effusion and aortitis: unusual main manifestations of giant cell arteritis].

Giant cell arteritis is a systemic vasculitis with segmentary vascular localisation, usually manifesting as temporal arteritis (Horton's disease). The predominant localisation in different vascular districts leads to clinical heterogeneity and poses a considerable diagnostic challenge. We describe a 77-year old woman with atypical presentation of giant cell arteritis, suffering from fever, weight loss and fatigue, but without classical symptoms such as polymyalgias, arthralgias and headache. The findings of pericardial effusion and thickening of aortic wall in chest-CT suggested the diagnosis of giant cell arteritis, fully confirmed by the following biopsy and histologic examination of a macroscopically and palpatory normal arteria temporalis. Undergoing an appropriate corticosteroid-medication the patient has been free of symptoms since 8 months.

Aged↗

Impending rupture of the ascending aorta due to giant cell arteritis.

Giant cell arteritis may occasionally lead to vessel perforation without previous dissection. At the level of the ascending aorta, however, such an event has been reported only three times. We report a fourth case of impending rupture of the ascending aorta due to a small and isolated lesion of giant cell arteritis. In contrast to previous reports, laboratory and clinical manifestations suggestive of giant cell arteritis were absent in this case so that the diagnosis could only be established on histologic examination of the operative specimen.

Aortic Rupture↗

[Giant cell arteritis].

Giant cell arteritis are characterized by histologic disruption features of the lamina elastica of large arteries with mononuclear cells and giant cells infiltration. Systemic giant cell arteritis, temporal arteritis and Takayasu's arteritis are included in this group. Takayasu's arteritis predominantly affects the teenage girl and involves the aorta and its mayor branches. Early manifestations are systemic; later appears signs and symptoms of occlusion.

Aortic Arch Syndromes↗

The implications of recognizing large-vessel involvement in elderly patients with giant cell arteritis.

Giant cell arteritis is associated with a markedly increased risk of aortic aneurysm formation or aortic rupture, particularly in the thoracic aorta. Although this association is not new, it has gained renewed attention recently. In this paper, the nature of aortic aneurysm formation in association with giant cell arteritis is discussed, along with the implications of recognizing large-vessel involvement in elderly patients with giant cell arteritis.

Aged↗

[Temporal arteritis (giant cell arteritis). Clinical picture, histology, and treatment].

Giant cell arteritis (arteritis temporalis) is the most common form of systemic vasculitis in the elderly. A series of symptoms such as new-onset headache, jaw claudication, proximal myalgia, weight loss, and fever may lead to the diagnosis. However, there is also a silent or occult presentation with minor or no systemic symptoms, especially no headache. A number of laboratory values (erythrocyte sedimentation rate, CRP, fibrinogen, thrombocytes, and cardiolipin antibodies) indicate giant cell arteritis, but none of this proves the diagnosis. Temporal artery biopsy is the gold standard for diagnosis of giant cell arteritis. Due to skip lesions, a negative result does not exclude the diagnosis. The most important complication of giant cell arteritis is visual loss in one or both eyes due to AION or retinal artery occlusion. Usually, visual loss is irreversible even with therapy. Corticosteroids are the drug of choice to treat giant cell arteritis. Therapy is required for a long time, monitored by parameters of inflammation (ESR, CRP).

Adrenal Cortex Hormones↗

Isolated renal giant cell arteritis.

Giant cell arteritis, which most commonly affects the temporal arteries, may involve intrarenal vessels and may be associated with a variety of renal lesions, including necrotizing arteritis, necrotizing glomerulonephritis, granulomatous glomerulonephritis, and membranous glomerulopathy. Isolated giant cell arteritis of the kidney is a rare cause of renal failure. We report a case of a previously healthy 54-year-old white woman who presented with nonoliguric renal failure and a 4-week history of persistent low-grade fever associated with diffuse mild myalgias. She had no history of previous renal or neurologic disease and denied any headaches or visual disturbances. Antinuclear antibody and antineutrophilic cytoplasmic antibody were negative. Renal biopsy revealed noncaseating granulomatous infiltration of arterial and arteriolar walls, a patchy mononuclear cell interstitial infiltrate, and no significant glomerular changes. Treatment with prednisone resulted in dramatic improvement of renal function.

Administration, Oral↗

Pulmonary artery obstruction due to giant cell arteritis.

Giant cell arteritis is often referred to in the context of polymyalgia rheumatica with temporal artery involvement. There are, however, more malignant forms of presentation of this necrotizing arteritis involving either the great vessels of the aorta or, occasionally, the pulmonary arteries. Our case relates to giant cell arteritis presenting as pulmonary artery obstruction in a patient without polymyalgia rheumatica or extensive aortic or proximal great vessel involvement.

Aged↗

Bilateral superficial femoral giant cell arteritis.

Giant cell arteritis is a rare, poorly understood, and often misdiagnosed entity. A case is reported of giant cell arteritis in a 30-year-old white male who developed severe bilateral peripheral claudication affecting both legs. The erythrocyte sedimentation rate (ESR) was markedly elevated. The diagnosis of vasculitis was established by histology postoperatively following exploration of both superficial femoral arteries and the placement of a reversed saphenous vein bypass graft to the right leg. Biopsy of the temporal artery revealed no pathology. The patient has been completely asymptomatic postoperatively and has resumed all previous normal activities. This condition has persisted in spite of a failed graft determined by an arteriogram performed 3 months after surgery. He has been treated with steroids continuously since the procedure.

Adult↗

Giant cell arteritis.

Giant cell arteritis has been considered an enigmatic disease. It is characterised by chronic granulomatous inflammation of the walls of large and medium-sized arteries. The process has a predilection for the extradural cranial arteries, which include the ophthalmic and the posterior ciliary arteries. It is a multi-symptom disease of older individuals and patients often present with challenging issues and diagnostic dilemmas. We review the literature and latest protocols for the diagnosis and management of giant cell arteritis.

Giant Cell Arteritis↗

Liver involvement in giant cell arteritis.

Giant cell arteritis is a vasculitis which usually affects large and medium-sized vessels in patients over 50 years old. The liver is one of the internal organs which can be involved in this systemic disease. During the last 15 years, 56 patients with giant cell arteritis were seen in our hospital. In 12 patients disturbed liver function test were found. In the majority of cases the disturbance was of cholestatic type and resolved completely with steroid treatment. The association of temporal arteritis with disturbed liver function tests is discussed, with a review of the recent literature.

Aged↗

[Horton's giant cell arteritis].

Giant cell arteritis (GCA) is a spontaneous vasculitic syndrome specifically involving the walls of medium and large arteries. While involvement of other arterial beds is occasionally identified, this syndrome is most frequently recognized when symptomatic involvement of the temporal arteries occurs. Vascular lesions are characterized by patchy granulomatous infiltrates composed of T cells, macrophages, histiocytes, and giant cells. A better prognosis depends on early recognition of the clinical symptoms and prompt treatment. Diagnosis was based on the 5 clinical criteria previously used by the American College of Rheumatology (1990): 1) age 50 years or older; 2) new localized headache; 3) temporal artery tenderness or decrease in temporal artery pulse; 4) erythrocyte sedimentation over 50 mm/ hour; 5) abnormal result on artery biopsy. Giant cell arteritis was considered a rare disease under age 50; however, it is now known to be an important and significant cause of morbidity and mortality in elderly people. Therefore early recognition and treatment with corticosteroid are very important. There is no general agreement concerning the initial dosage, 40-65 mg/day are commonly recommended. After a few months the majority of patients can be treated with a low maintenance dosage of prednisolone (5 to 7.5 mg/day). The mean duration of treatment is about 5 years. The literature is reviewed and the clinical implications of this disease are discussed.

Giant Cell Arteritis↗

Giant cell arteritis.

Giant cell arteritis is a form of vasculitis affecting medium- and large-size arteries, with a predilection for vessels arising from the aortic arch. The origins of giant cell arteritis are unknown. However, ethnic affinities are prominent, with the majority of cases involving white, elderly patients. In addition, there is evidence of a familial pattern and an association with HLA-DR4, suggesting a genetic predisposition in select cases. With an increase in the elderly population, clinicians must become cognizant of this age-related malady and its treatment to ensure quality care for elderly patients.

Adrenal Cortex Hormones↗

Recovery of vision in a 47-year-old man with fulminant giant cell arteritis.

Giant cell arteritis is a systemic necrotizing vasculitis that often causes profound and irreversible visual loss in elderly individuals. We describe a 47-year-old man with fulminant giant cell arteritis whose clinical picture included severe visual loss and several unusual or previously unreported findings. Aggressive treatment with intravenous corticosteroids resulted in a dramatic improvement in the patient's vision. Although no firm conclusions can be drawn from the outcome in a single case, we believe that, in some patients with arteritic ischemic optic neuropathy, aggressive treatment with intravenous corticosteroids may be associated with a better visual prognosis than treatment by the oral route.

Giant Cell Arteritis↗

Role for vascular investigations in giant cell arteritis.

Giant cell arteritis is characterized by diffuse arterial inflammation that selectively involves the superficial temporal arteries but can occur in larger arteries. Various vascular investigations can assist in diagnosing and evaluating the extent of giant cell arteritis. Imaging techniques, mainly Doppler ultrasonography of the superficial temporal arteries, seem less reliable for the diagnosis than temporal artery biopsy, which is safe and remains indispensable. Investigations of larger arteries can detect asymptomatic stenotic lesions, which are common, particularly in the axillary and subclavian arteries. Involvement of the aorta can cause life-threatening dissection or aneurysmal rupture. Imaging techniques useful for diagnosing aortic involvement include ultrasonography, computed tomography, magnetic resonance imaging, and aortography. Although there is no standardized strategy for aortic lesion detection, helical computed tomography may be valuable.

Aortitis↗