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Query: UMLS:C0018681 (headache)
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Cranial arteritis (CA), also called giant cell arteritis or temporal arteritis, is a vasculitis primarily affecting adults over age 50. It is a large vessel vasculitis, and giant cells classically can be identified on histopathologic examination of temporal arteries, but are not essential for diagnosis. Patients typically present with severe headaches, fatigue, polymyalgia-like symptoms, or ischemic complaints such as jaw claudication. Visual loss is the major feared irreversible outcome and can occur in up to 50% of those with untreated disease. Glucocorticoids, typically high dose prednisone (> or = 60 mg/d) is the first-line treatment and successfully controls the inflammatory disease in the vast majority of patients. Most patients can be tapered off steroids within 6 months to 2 years.
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PMID:Inflammatory disease in older adults. Cranial arteritis. 1566 19

Temporal arteritis, including large-vessel giant cell arteritis, and Takayasu's arteritis are the two primary large-vessel vasculitides. Patients with temporal arteritis often present with headache, swollen temporal arteries, impairment of vision or symptoms of polymyalgia rheumatica. Clinical examination includes palpation of the temporal arteries and radial pulses, auscultation of the subclavian and axillary region, and fundoscopy. The presence of jaw claudication, diplopia and temporal artery abnormalities correlates with a high probability of positive histology. Duplex ultrasonography of the temporal arteries delineates a characteristic hypoechoic, oedematous wall swelling, stenoses and occlusions. It detects the same pathologies in the axillary arteries and other arteries in large-vessel giant cell arteritis. Angiography, magnetic resonance imaging, magnetic resonance angiography, electron beam computed tomography, computed tomography angiography and positron emission tomography show characteristic changes in the aorta and its primary branches in large-vessel giant cell arteritis and Takayasu's arteritis. Takayasu's arteritis often begins with diffuse symptoms such as low-grade fever, arthralgia, fatigue and weight loss. Clinical examination is important to detect bruits, pulse reduction and blood pressure differences. Profound experience exists with angiography. Other imaging methods are interesting alternatives as they are less invasive and may depict the inflammatory wall swelling.
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PMID:What is the best approach to diagnosing large-vessel vasculitis? 1585 93

Polymyalgia rheumatica (PMR) and giant cell arteritis (GCA) are closely related and frequently occurring inflammatory diseases with an incidence of 50 and 18 per 100,000 per year, respectively, in people aged 50 years or over. The most frequent symptom of PMR is aching and morning stiffness lasting more than 1 month and exacerbated by movement, occurring in the shoulder and pelvic girdles and in the neck region. GCA is vasculitis of the large and medium-sized arteries that originate from the aortic arch, causing new and marked headache localised over the temporal or occipital areas, jaw claudication, visual impairment or claudication of the arms. GCA is characterised by histopathological panarteritis with a predominantly lymphohistiocytic cell infiltrate. Activation of macrophages is central to the arteritis. Standard treatment for PMR and GCA is glucocorticoids, which may consist of prednisone 10-20 mg/day or its equivalent for PMR patients and prednisone 30-40 mg to 1 mg/kg body weight for GCA patients. For GCA patients with recently impaired vision, treatment should start with high doses of intravenously administered glucocorticoids, such as methylprednisolone 1 g/day for 3 consecutive days. A treatment duration of 1-2 years is often required for patients with PMR or GCA; because of the side effects associated with long-term use of glucocorticoids, osteoporosis prophylaxis with oral calcium supplementation, vitamin D and bisphosphonates is appropriate.
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PMID:[Polymyalgia rheumatica and temporal arteritis]. 1630 94

Middle aortic syndrome (MAS) is a clinical condition generated by segmental narrowing of the abdominal or distal descending thoracic aorta. MAS may be acquired, caused by Takayasu's or temporal arteritis (giant cell arteritides), neurofibromatosis, fibromuscular dysplasia, retroperitoneal fibrosis, mucopolysaccharidosis, and the Williams syndrome, or congenital, ascribed to a developmental anomaly in the fusion and maturation of the paired embryonic dorsal aortas. Segmental aortic stenosis may be located at the suprarenal, inter-renal or infrarenal aorta, with a high propensity for concomitant stenoses in both the renal (63%) and visceral (33%) arteries. Hypertension proximal to the aortic stenosis, and relative hypotension distal to it, are characteristic findings in MAS. Typical manifestations include headache, early fatigue on exertion, and bilateral lower-limb claudication. The severity of hypertension is the primary indication for intervention and the factor determining procedural timing. As a great proportion of patients with MAS are children or teenagers, the clinical benefits of early surgical intervention to reverse refractory hypertension have to be weighed against the repercussions pertaining to the insult of surgery on the developing aorta. Open surgery is the primary treatment of tubular aortic narrowing (MAS) associated with renovascular hypertension and visceral artery stenosis. This entails aortoaortic bypass of the diseased segment or, less often, patch aortoplasty and usually bypass grafting of the stenosed renal and visceral arteries performed with autologous conduits, particularly in the youngest of patients. Endovascular therapy may provide a sound minimally invasive treatment in MAS caused by discrete aortic stenoses that do not encompass the mesenteric and renal arteries. Hypertension is thus improved or cured in more than 70% of patients. Prognosis after uncompromised surgical reconstruction is rewarding in the mid and long term in patients with congenital aortic coarctation but deteriorates in patients with aortoarteritis and recurrent inflammatory activity.
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PMID:Middle aortic syndrome: from presentation to contemporary open surgical and endovascular treatment. 1627 55

Giant cell arteritis (GCA) is well known for its involvement of the proximal aorta and its branches, classically causing headache, visual impairment, and elevations in the erythrocyte sedimentation rate (ESR) or C-reactive protein (CRP). We describe a case of biopsy-proven GCA initially presenting with limb claudication, oligoarticular inflammatory arthritis, and a positive antineutrophil cytoplasmic antibody with cytoplasmic staining (C-ANCA), treated successfully with a combination of prednisone and weekly methotrexate. This case illustrates the wide spectrum of features that can be seen with GCA, including the occasional presence of C-ANCA. The C-ANCA became negative after treatment.
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PMID:Disseminated giant cell arteritis with inflammatory arthritis and C-ANCA. 1635 60

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).
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PMID:[Temporal arteritis (giant cell arteritis). Clinical picture, histology, and treatment]. 1655 35

Temporal arteritis is a rheumatic disease that affects large and medium-sized arteries. It is a severe arteritis involving both the intima and media of the vessel and is a cause of headache that is frequently diagnosed erroneously as "atypical migraine." The patients have a burning or throbbing type of pain. Ultimately, there is localized inflammation or cellulitis over the swollen, tortuous artery. Jaw claudication, eye pain, photophobia, diplopia, and even blindness may accompany the temporal symptoms. As many as 20% to 60% of inadequately treated or untreated patients will lose their vision. Blindness may or may not be preceded by visual symptoms and funduscopic changes. A variety of systemic symptoms are also often present, including nausea, vomiting, chills, dizziness, and loss of weight. Temporal arteritis is not a common diagnosis in maxillofacial practice. We are presenting a case of temporal arteritis diagnosed after a biopsy. The patient eventually lost the vision from one eye.
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PMID:Temporal arteritis: report of a case. 1687 61

Polymyalgia rheumatica and giant cell arteritis are common, closely related vasculitic conditions that almost exclusively occur in patients older than 50 years. They may be manifestations of the same underlying disease and often coexist. Patients with polymyalgia rheumatica usually present with acute onset of stiffness and pain in the shoulder and pelvic musculature, which may be accompanied by fever, malaise, and weight loss. If untreated, polymyalgia rheumatica may result in significant disability. Giant cell arteritis may manifest as visual loss or diplopia, abnormalities of the temporal artery such as tenderness or decreased pulsation, jaw claudication, and new-onset headaches. Erythrocyte sedimentation rate and temporal artery biopsy help make the diagnosis. Giant cell arteritis requires urgent diagnosis because without treatment it may lead to irreversible blindness. Patients with either condition also may have nonspecific symptoms. Corticosteroids are the mainstay of therapy for both conditions, with higher doses required for treatment of giant cell arteritis. Duration of corticosteroid therapy can be five years or longer before complete clinical remission is achieved. Monitoring for corticosteroid-associated side effects such as osteoporosis and diabetes, as well as for relapses and flare-ups, is key to chronic management. The prognosis for either condition, if treated, is good.
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PMID:Polymyalgia rheumatica and giant cell arteritis. 1711 94

The author set out to review the thought processes of Bayard Horton as he was clinicopathologically describing the first cases of temporal arteritis. The Mayo Clinic records of the original temporal arteritis patients were examined. Horton obtained the first biopsies of the temporal arteries in temporal arteritis and was the first to describe the histopathology. Horton initially thought his first two patients had actinomycosis of the temporal arteries, but later abandoned this diagnosis. He reported these two patients in 1932 as 'an undescribed form of arteritis of the temporal vessels'. He was the first to describe jaw claudication. He saw a patient with blindness and symptoms suggestive of temporal arteritis before this complication was described in the literature, but initially felt the patient had some other disease. The sedimentation rate was elevated in his first patient. He cared for the first temporal arteritis patient ever treated with cortisone.
Cephalalgia 2007 Jan
PMID:Bayard Horton's clinicopathological description of giant cell (temporal) arteritis. 1721 86

Giant cell arteritis (GCA) is the most common vasculitis in Western countries in individuals over the age of 50. The diagnosis is relatively straightforward when typical features, such as headache, jaw claudication or other ischemic complications are present. Although atypical presentations of GCA have been described, herein we report for first time low back pain as the presenting manifestation of this vasculitis. We also emphasize the importance of considering the use of positron emission tomography (PET) in the evaluation of GCA patients presenting without "overt" cranial ischemic manifestations.
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PMID:Low back pain as presenting manifestation of giant cell arteritis associated to abdominal aortitis. 1742 61


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