Giant-Cell Arteritis and Polymyalgia Rheumatica
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Abstract:
Both giant-cell arteritis and polymyalgia rheumatica are immune-mediated diseases that are treated with glucocorticoids, with higher doses used for giant-cell arteritis. Prompt initiation of high doses and a biopsy are recommended when ischemic optic neuropathy is suspected.Keywords:
Polymyalgia rheumatica
Arteritis
Background. Giant cell arteritis is a vasculitis affecting large- and medium-calibre vessels. It is not uncommon in the West and there are many large series in the literature. However, there are very few reports of giant cell arteritis among Indian patients. Methods. We did a retrospective study of 9 Indian patients (5 men and 4 women; age range 59–81 years [mean and median 70 years]) who had had a temporal artery biopsy for suspected giant cell arteritis at a tertiary care hospital. Results. Eight patients had biopsy-proven giant cell arteritis. The common presenting features were pyrexia of unknown origin (4), headache (6) and blurring of vision (2). The erythrocyte sedimentation rate was elevated and ranged from 25 to 120 mm at the end of the first hour (mean 96, median 105). The C-reactive protein level, which was available in 5 cases, was raised. Giant cells and inflammatory cells were seen in 7 of 8 temporal artery biopsies; a transmural lymphocytic and neutrophil infiltrate without giant cells was present in 1 case. All patients were treated with steroids and they responded well. Conclusion. Temporal arteritis is probably under-recognized in India. Pyrexia is a common presenting feature of the disease; temporal arteritis should be considered in the differential diagnosis of elderly patients with pyrexia of unknown origin. Natl Med J India 2010;23:18–20
Erythrocyte sedimentation rate
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Polymyalgia rheumatica
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Systemic vasculitis
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Giant cell arteritis (GCA) is a serious disease and the most common cause of vasculitis in the elderly. Here, studies describing the recent discovery of varicella zoster virus (VZV) in the temporal arteries of patients with GCA are reviewed.GCA is characterized by severe headache/head pain and scalp tenderness. Many patients also have a history of vision loss, jaw claudication, polymyalgia rheumatica, fever, night sweats, weight loss, and fatigue. The erythrocyte sedimentation rate and C-reactive protein are usually elevated. Diagnosis is confirmed by temporal artery biopsy, which reveals vessel wall damage and inflammation, with multinucleated giant cells and/or epithelioid macrophages. Skip lesions are common. Importantly, temporal artery biopsies are pathologically negative in many clinically suspect cases. The present review highlights recent virological findings in temporal arteries from patients with pathologically verified GCA and in temporal arteries from patients who manifest clinical and laboratory features of GCA but whose temporal artery biopsies are pathologically negative for GCA. Virological analysis revealed that VZV is present in most GCA-positive and GCA-negative temporal artery biopsies, particularly in skip areas that correlate with adjacent GCA disease.The presence of VZV in GCA-positive and GCA-negative temporal arteries reflects the possible role of VZV in triggering the immunopathology of GCA and indicates that both groups of patients should be treated with antivirals in addition to corticosteroids. Whether oral antiviral agents and steroids are as effective as intravenous acyclovir and steroids, and the dosage and duration of treatment, remain to be determined.
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To study the possible contribution of fluorodeoxyglucose (FDG)-positron emission tomography (PET) in the diagnosis of giant cell arteritis and polymyalgia rheumatica.A consecutive case series consisting of five patients with polymyalgia rheumatica, six patients with temporal arteritis and 23 age-matched patients with other inflammatory conditions were evaluated with FDG-PET. Studies were performed before therapy with steroids was started.A total of 4/6 patients with giant cell arteritis and 4/5 patients with polymyalgia had increased FDG uptake in their thoracic vessels, compared to 1/23 controls (P < 0.001). Increased vascular FDG uptake in the upper legs was seen in 8/11 patients with giant cell arteritis or polymyalgia compared to 8/23 control patients (P < 0.05), and in the lower legs in 6/11 patients compared to 6/23 controls (P = not significant).FDG-PET scan is the first non-invasive technique which may indicate large-vessel vasculitis and which can show its extension throughout the body. It strongly suggests that polymyalgia rheumatica is a form of vasculitis.
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Abstract: Giant cell arteritis is the most common vasculitis in Caucasians. Acute visual loss in one or both eyes is by far the most feared and irreversible complication of giant cell arteritis. This article reviews recent guidelines on early recognition of systemic, cranial, and ophthalmic manifestations, and current management and diagnostic strategies and advances in imaging. We share our experience of the fast track pathway and imaging in associated disorders, such as large-vessel vasculitis. Keywords: giant cell arteritis, diagnosis, treatment, polymyalgia rheumatica
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Giant cell arteritis (GCA) is an inflammatory vasculitis of unknown etiology that mainly involves large and medium arteries, particularly the cranial branches of the aorta. GCA with consecutive arteritic-anterior ischemic optic neuropathy (A-AION) has rarely been diagnosed in Romania. Recently, we encountered an 83-year-old patient who presented with left eye visual impairment and corresponding optic disc diffusely swollen and pale. He also had typical manifestations of GCA, such as malaise, and temporal headache, and a highly elevated erythrocyte sedimentation rate and C-reactive protein level. Biopsy of his left superficial temporal artery revealed a granulomatous inflammation with multinucleated giant cell infiltration, so he was diagnosed with GCA with consecutive left A-AION. Because without treatment, this affection usually progresses very rapidly, the patient was promptly treated with an adequate dosage of steroids, which was essential to save the visual function of both eyes. Our case report confirms the potential of visual recovery after prompt corticosteroid treatment in GCA with eye involvement.
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We describe a heterogeneous group of 4 elderly patients with atypical vasculitis of the temporal artery who presented clinically with the polymyalgia rheumatica-temporal arteritis syndrome. The first had apparently isolated eosinophilic necrotizing vasculitis without peripheral blood eosinophilia, history of asthma, or pulmonary vasculitis (so called limited form of Churg-Strauss syndrome). Temporal artery biopsy in the 2nd patient revealed small vessel necrotizing vasculitis in the fragment of skeletal muscle surrounding a normal temporal artery (i.e., negative for giant cell arteritis, GCA). This patient subsequently developed rheumatoid polysynovitis. The temporal artery biopsies in the remaining 2 patients showed vasa vasorum vasculitis, without GCA, associated with malignancy in Patient 3 and as an unexpected and apparently isolated finding in Patient 4. All 4 patients responded to the standard corticosteroid therapy for giant cell temporal arteritis. Unusual temporal artery biopsies such as these should be interpreted in relation to clinical findings.
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Giant cell arteritis (GCA) is a granulomatous medium to large vessel vasculitis. It typically occurs in older people and is associated with polymyalgia rheumatica (PMR).[1][1] The condition usually manifests with involvement of the extracranial branches of the carotid artery. This results in the
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