Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0039483 (giant cell arteritis)
 3,204 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Among the population of Olmsted County, Minnesota, 42 patients with temporal arteritis were identified during a 25-year period. The average annual incidence per 100 000 population aged 50 and older rose from 5.1 in 1950-1959 to 17.4 in 1970-1974. The prevalence of patients with a history of the diagnosis of temporal arteritis on 1 January 1975 was 133 per 100 000 population aged 50 and older. All patients received corticosteroid therapy for a range of 1 to 77 months (median, 7 months). Relapses in 10 of 11 patients were associated with corticosteroid reduction. The majority of patients recovered fully and were followed off corticosteroids for 10 months to 19 years (median, 5 years). Temporal arteritis had no significant effect on survival. Vertebral compression fractures and myopathy were the most serious complications of therapy. The presence of giant cells in biopsies was in part related to the number of sections examined, and their presence had no apparent influence on the clinical course.
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PMID:Temporal arteritis: a 25-year epidemiologic, clinical, and pathologic study. 62 44

The analysed clinico-biological manifestations, evolutive course and treatment of 30 patients with GCA are presented. The most frequent symptoms were fever and headache. 33% of patients had FOD criteria. 26% had various visual alterations. All patients were initially treated with steroids. Of the 26 patients followed up, 21 (81.7%) experienced some sort of complication: Cushing iatrogenic, osteoporosis, vertebrae collapse, aseptic necrosis of the femur head, arterial hypertension, diabetes mellitus, hyperlipidemia, steroid myopathy. 6 patients were treated with cyclophosphamide, following severe complications secondary to steroid therapy, and all of them had a good clinical evolution.
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PMID:[Giant-cell arteritis: the clinico-biological manifestations and the complications secondary to steroid treatment]. 191 67

The atypical clinical course of giant-cell arteritis in the elderly (who may develop a clinical picture of severe consumptive disease) is illustrated by two observations with histologically confirmed temporal arteritis. In addition to fever and loss of weight, the inflammatory vascular process in a 78-year old female was reflected in arrhythmias (atrial fibrillation and atrial flutter), probably due to involvement of the coronary arteries, and occlusion of the left axillary artery. Similar general symptoms and various neurological deficits comprising amaurosis, mononeuritis multiplex, polyneuropathy, myopathy and finally subarachnoid hemorrhage characterized the disease in a 72-year-old man. The picture was further complicated by intestinal perforation. In both patients steroids brought considerable improvement and the disease process came to a standstill.
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PMID:[Atypical giant cell arteritis]. 199 Apr 21

We report a prospective study of 42 patients with biopsy-proven giant cell arteritis (CGA) who recovered for more than one year (mean follow up of 71 months since withdrawal of steroid treatment). It was used the same regimen of prednisone and well closely monitored along the whole treatment. In 22 patients, dapsone was given concomitantly with prednisone. Mean duration of steroid therapy was 23.1 months (range: 6-57 months); it was significantly decreased with treatment by dapsone (12 months and 12 days). Age, sex, initial clinical and biological (acute phase reactants) findings did not provide useful information for predicting steroid treatment needed for recovery. Thirty-six relapses were observed in 22 patients (60%) during treatment or after its withdrawal. Incidence of relapses declined during steroid treatment and relapses were (only) observed over first 6 months after steroid withdrawal. Three amaurosis fugax occurred at the beginning of treatment and an axillar bilateral stenosis was also observed. Forty-eight side effects of corticosteroids were recorded in 26 patients (63%): myopathy (n = 12), bone complications (n = 11), metabolic complications (n = 9). Twelve patients (63%) had experienced side effects of dapsone. This study emphasized the difficulty in treating CGA. Close monitoring is required. A steroid regimen is recommended.
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PMID:[Clinical course of Horton disease. Apropos of 42 cured patients]. 809 45

Polymyalgia rheumatica (PMR) is a disease of unknown aetiology that occurs in elderly patients, predominantly affecting the Caucasian population. The disease has a slightly higher prevalence in women than in men. There is ongoing discussion regarding the relationship between PMR and giant cell arteritis; an increasing number of studies indicate that they are closely related. PMR has also been linked with rheumatoid arthritis, myopathy and malignant disease. Oral corticosteroids remain the mainstay of drug therapy for PMR. These drugs usually induce prompt relief of symptoms, and some authors consider this dramatic response to be diagnostic for PMR. However, the ideal initial dosage, the duration of treatment and the optimal tapering schedule are much debated. Other drugs, such as methotrexate and azathioprine, have been suggested as corticosteroid sparing agents. Nonsteroidal anti-inflammatory drugs are generally considered to be unsuitable for the long term treatment of PMR.
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PMID:Epidemiology and optimal management of polymyalgia rheumatica. 973

Oculopharyngeal muscular dystrophy (OPMD) is an adult-onset autosomal dominant myopathy found world-wide, but with the highest incidence in French-Canadians. Short GCG expansions in the poly(A) binding protein 2 (PABP2) gene were identified recently as the molecular basis for OPMD in French-Canadians. All French-Canadian cases of OPMD have been traced to a single founder couple [Bouchard, 1997: Neuromuscul Disord 7(Suppl):S5-S11]. Cultural links between French-Canadians and Cajuns suggest that this same founder couple may have transmitted the OPMD mutation to Cajuns as well. To determine if OPMD patients from Louisiana share a founder effect with French-Canadian families, we collected blood samples and muscle biopsies from several Cajuns with OPMD for mutation and linkage studies. We found a unique 'GCA GCG GCG' insertion mutation in Cajuns. Consistent with these sequence data, we identified a disease haplotype in our Cajun families that is different from the ancestral haplotype defined in French-Canadians. These data prove that different founders introduced the PABP2 mutation to Cajuns and French-Canadians and lend support to emerging genealogical data suggesting that French-Canadians and Cajuns represent distinct immigrant groups from France.
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PMID:Unique PABP2 mutations in "Cajuns" suggest multiple founders of oculopharyngeal muscular dystrophy in populations with French ancestry. 1050 91

A case of giant cell arteritis with systemic and panocular involvement is reported here. This elderly Indian male presented with symptoms of unilateral temporal headache and intermittent jaw claudication for a month followed by diplopia and blurring of vision and later loss of vision in the right eye. The right eye showed some limitation of ocular movements, presence of relative afferent pupil defect, anterior segment ischaemic changes and anterior ischaemic optic neuropathy. Visual evoked potential showed an absent P1 wave while the left eye with normal 6/6 vision sowed a prolonged P1 wave. Fundus fluoresceine angiography showed delay in choroidal perfusion. His erythrocyte sedimentation rate (ESR) was 120 mm/hr and he was started on oral prednisolone. Superficial temporal artery biopsy obtained one week after starting steroids was positive for giant cell arteritis. Steroids led to the resolution of optic disc swelling, disappearance of anterior segment signs, full recovery of right ocular movements and no further deterioration of the fellow eye. On steroids, he developed insomnia and progressive myopathy which resolved and is now symptom free at lower doses of steroids.
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PMID:Giant cell arteritis with panocular involvement in an Indian male. 1455 35

Polymyalgia rheumatica (PMR) typically manifests as inflammatory pain in the shoulder and/or pelvic girdles in a patient over 50 years of age. This condition was long underrecognized and therefore underdiagnosed. Today, however, overdiagnosis may occur. Physicians must be aware that many conditions may simulate PMR, including diseases that carry a grim prognosis or require urgent treatment. PMR may be the first manifestation of giant cell arteritis, and a painstaking search for other signs is mandatory. PMR may inaugurate other rheumatologic diseases such as rheumatoid arthritis, RS3PE syndrome, spondyloarthropathy, systemic lupus erythematosus (SLE), myopathy, vasculitis, and chondrocalcinosis. Finally, PMR may be the first manifestation of an endocrine disorder, a malignancy, or an infection. Failure to respond to glucocorticoid therapy should suggest giant cell arteritis, malignant disease, or infection. Ultrasonography may assist in the diagnosis by showing bilateral subdeltoid bursitis. Glucocorticoids are the mainstay of the treatment of PMR. Although the optimal starting dosage and tapering schedule are not agreed on, a low starting dosage and slow tapering may decrease the relapse rate. Methotrexate is probably useful when glucocorticoid dependency develops. In contrast, TNF-alpha antagonists are probably ineffective.
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PMID:Polymyalgia rheumatica: diagnosis and treatment. 1711 8

Oculopharyngeal muscular dystrophy (OPMD) is a late-onset muscle disorder characterized by progressive dysphagia and bilateral ptosis. Mutations in the polyadenylate binding protein nuclear 1 (PABPN1) gene have been found to cause OPMD. The typical mutation is a stable trinucleotide repeat expansion in the first exon of the PABPN1 gene, in which (GCG)(6) is the normal repeat length. We investigated a Korean patient with OPMD and identified a novel mutation: a heterozygous insertion of a 9-bp sequence [(GCG)(GCA)(GCA); c.27_28insGCGGCAGCA] instead of the (GCG) repeat expansion, resulting in an in-frame insertion of three alanines (p.A10insAAA). To the best of our knowledge, this is the first report of a genetically confirmed case of OPMD in Korea.
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PMID:Identification of a novel mutation in a Korean patient with oculopharyngeal muscular dystrophy. 1713 75

Ipilimumab is a novel humanized monoclonal antibody directed against cytotoxic T lymphocyte antigen 4, a T-cell surface molecule involved in down-regulation and suppression of the T cell response to stimuli. Patients treated with ipilimumab are at risk for immune-related adverse events involving the skin, digestive tract, liver and endocrine organs. Few case reports of immune-related adverse effects involving central or peripheral nervous system due to ipilimumab are published. These include inflammatory myopathy, aseptic meningitis, severe meningo-radiculo-neuritis, temporal arteritis, Guillain-Barre syndrome, and posterior reversible encephalopathy syndrome. We report the first case of ipilimumab-induced progressive necrotic myelopathy.
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PMID:Ipilimumab-induced necrotic myelopathy in a patient with metastatic melanoma: A case report and review of literature. 2571 27


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