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1.
Giant cell arteritis (GCA) is a medium and large‐vessel vasculitis, which is an important cause of secondary headache in older adults. While GCA has a classic presentation occurring after the age of 50, atypical presentations (eg, fever of unknown origin, cough, low or normal erythrocyte sedimentation rate) may lead to a delay in diagnosis. The topography of vascular involvement has implications for disease‐related complications, which can result in neurologic disease at multiple levels of the nervous system. The most feared complication, vision loss, fortunately becomes uncommon after initiation of corticosteroids. Corticosteroid treatment should not be withheld while waiting the results of a temporal artery biopsy (TAB), which remains the gold standard for GCA diagnosis. Newer diagnostic modalities, including ultrasound, magnetic resonance imaging, and positron emission tomography can play an important role in directing treatment in cases with negative TAB. After successful control of the disorder, patients should be gradually tapered off corticosteroids, with careful monitoring using both clinical and laboratory parameters to assess for relapse. Corticosteroid‐related treatment complications are not uncommon in GCA. There is mixed evidence for use of adjunct corticosteroid‐sparing agents (eg, methotrexate), although these should be initiated in the setting of corticosteroid‐related morbidity and/or cases with frequent relapse.  相似文献   

2.
Polymyalgia rheumatica (PMR) was defined in 1957 and is linked with giant cell arteritis (GCA) in approximately 25% of cases. The peak incidence is between 60 and 75 years old and is increasing with the ageing population. Polymyalgia rheumatica is a clinical diagnosis without a 'gold standard' serological or histological test and there are other conditions that may mimic PMR. Treatment with a dose of 10-20 mg daily of prednisolone is suggested or 40-60 mg daily if GCA is also suspected. There are no absolute guidelines to the dose or its duration. The rate of reduction should be adjusted depending on the individual's response. Where temporal arteritis is suspected, this manifestation of GCA is a treatable medical emergency to prevent possible blindness, and steroids should be commenced immediately. There remain many unknowns in the cause, diagnosis and treatment of PMR and its overlap with GCA, and it is an ongoing challenge requiring further research.  相似文献   

3.
ObjectiveTo compare and rank the effect of glucocorticoid-sparing agents in giant cell arteritis (GCA), for which several drugs have been evaluated but with a benefit-risk balance that remains uncertain.MethodsThe MEDLINE and Clinical Trials databases were searched up to November 2021; all randomized controlled trials investigating glucocorticoids in GCA were included. The glucocorticoid regimen was dichotomized into short (≤6 months) or prolonged (>6 months) use. Risk of relapse and safety were estimated using network meta-analysis with frequentist random effects models.ResultsOf the 96 records screened, 8 trials were included (572 patients). The trials compared glucocorticoids and a sparing agent: tocilizumab (2 trials), oral methotrexate (3 trials), infliximab (1 trial), etanercept (1 trial), and adalimumab (1 trial). The pooled prevalence of GCA relapse was 52.6% (95% CI, 38.1 to 66.9). The risk of relapse was significantly lower with tocilizumab compared with methotrexate (relative risk [RR], 0.41; 95% CI, 0.17 to 0.97) and prolonged (RR, 0.41; 95% CI, 0.20 to 0.83) and short (RR, 0.32; 95% CI, 0.16 to 0.66) glucocorticoid use. The risk of relapse was not significantly different with methotrexate compared with short (RR, 0.79; 95% CI, 0.48 to 1.31) and prolonged (RR, 0.95; 95% CI, 0.31 to 2.89) glucocorticoid use. The frequency of serious adverse events and serious infection was comparable between the different drugs. The certainty of the evidence was low to very low.ConclusionThis meta-analysis suggests that tocilizumab may be superior to other sparing agents to prevent GCA relapse, but with a low to very low certainty of evidence, and that safety is comparable to the other drugs.RegistrationThe protocol of the meta-analysis is registered in the international prospective register of systematic reviews PROSPERO (https://www.crd.york.ac.uk/prospero/; registration CRD42020112387).  相似文献   

4.
Introduction: Giant cell arteritis is a vasculitis of large and middle-sized arteries that affects individuals older than 50 years. Although glucocorticoids remain the mainstay in the treatment of this vasculitis, other drugs are often required to achieve clinical remission and allow glucocorticoid discontinuation.

Areas covered: The review summarizes the main biologic therapies used for the managements of GCA.

Expert commentary: Although several biologic agents have been used in patients with GCA, the only biologic agent currently approved for this purpose is the recombinant humanized anti-IL-6 receptor antibody: tocilizumab. It has demonstrated efficacy to improve clinical symptoms, decrease the cumulative prednisone dose and reduce the frequency of relapses in clinical trials and real-life studies on patients with GCA. A trial showed that abatacept may be useful to maintain remission in GCA patients. An open-label study suggested that ustekinumab could be useful for the treatment of patients with refractory GCA. However, further studies are required to confirm if both abatacept and ustekinumab are useful as an adjunctive therapy to reduce relapses or as a glucocorticoid‐sparing agent in GCA. Anakinra has been successfully used in a few patients with refractory GCA. In contrast, antitumor necrosis factor-α therapy yielded disappointing results in GCA.  相似文献   


5.
Giant cell arteritis (GCA) is a granulomatous and occlusive vasculitis that causes blindness, stroke, and aortic aneurysm. CD4(+) T cells are selectively activated in the adventitia of affected arteries. In human GCA artery-severe combined immunodeficiency (SCID) mouse chimeras, depletion of CD83(+) dendritic cells (DCs) abrogated vasculitis, suggesting that DCs are critical antigen-presenting cells in GCA. Healthy medium-size arteries possessed an indigenous population of DCs at the adventitia-media border. Adoptive T cell transfer into temporal artery-SCID mouse chimeras demonstrated that DCs in healthy arteries were functionally immature, but gained T cell stimulatory capacity after injection of lipopolysaccharide. In patients with polymyalgia rheumatica (PMR), a subclinical variant of GCA, adventitial DCs were mature and produced the chemokines CCL19 and CCL21, but vasculitic infiltrates were lacking. Human histocompatibility leukocyte antigen class II-matched healthy arteries, PMR arteries, and GCA arteries were coimplanted into SCID mice. Immature DCs in healthy arteries failed to stimulate T cells, but DCs in PMR arteries could attract, retain, and activate T cells that originated from the GCA lesions. We propose that in situ maturation of DCs in the adventitia is an early event in the pathogenesis of GCA. Activation of adventitial DCs initiates and maintains T cell responses in the artery and breaks tissue tolerance in the perivascular space.  相似文献   

6.
Polymyalgia rheumatica (PMR) is a disease of unknown etiology characterized by severe myalgia and stiffness at shoulder girdle and pelvic girdle muscles and by normal serum creatine kinase levels. Marked elevation of erythrocyte sedimentation rate, acute onset within two weeks, and appearance in the aged are also additional characteristics of PMR. Ten to 50% of PMR patients have a concomitant temporal arteritis (TA)(giant cell arteritis). For the differential diagnoses of PMR, rheumatoid arthritis, polymyositis, fibromyalgia, malignancies, infections and depression should be considered. PMR without TA is treatable successfully with small amount of steroids (15-20 mg/day of prednisolone). For the PMR patients with TA should be treated with large amount of steroids (40-60 mg/day of prednisolone) or steroid pulse therapy.  相似文献   

7.
尽管治疗的进步极大地改善了急性白血病患者的预后和生存,但时至今日多数类型的急性白血病没有特异性的生物标记,因此对于多数白血病患者,影响生存的复发这一重要因素缺少有效的预警机制。删基因启动子区甲基化广泛发生于各类型急性白血病。本研究在前期建立的甲基化定量PCR体系的基础上,用该方法检测患者骨髓样本,探讨ID4甲基化定量指标(percentageofmethylatedreference,PMR)的临床意义。采集我院门诊及住院确诊的初治、完全缓解、复发3个阶段的急性白血病患者骨髓样本及正常对照者骨髓样本。应用ID4甲基化定量PCR体系对样本进行检测。按初治、完全缓解、复发分组比较PMR值。比较相同病例不同疾病状态的PMR的动态变化。结果表明,初治组PMR最高,其次为复发组,而完全缓解组最低。初治组PMR与完全缓解组比较存在统计学差异。4例随访病例的PMR值波动与病情变化一致。在1例复发病例中,PMR升高早于骨髓细胞学检查确认复发1.7个月。结论:本研究通过1/94基因启动子区甲基定量检测的方法初步验证:甲基化水平的量化指标PMR值与急性白血病患者肿瘤细胞负荷关系密切。PMR动态监测波动与疾病变化一致,可能具有预测复发的作用,但IIM甲基化定量指标的临床价值还有待进一步研究证据的支持。  相似文献   

8.
BACKGROUND: Systemic small-vessel vasculitis (SVV) is increasing in incidence and age of diagnosis. Presenting features may mimic those of polymyalgia rheumatica (PMR), a common disease of the elderly. Aim: To test the hypotheses that SVV is frequently misdiagnosed as PMR in elderly patients, that this results in a delay in diagnosis and appropriate treatment, and that the natural history and clinical features are different. DESIGN: Retrospective case-control study. METHODS: Cases of glomerulonephritis due to SVV at a single centre over a 12-year period were analysed, comparing those treated previously for PMR (PMR(+)) to the remainder of the cohort (PMR(-)). RESULTS: Of 86 patients with complete follow-up, 13% had been treated previously for PMR. PMR(+) patients had a longer duration of symptoms prior to SVV diagnosis (396 vs. 107 days, p = 0.001) and were less likely to be dialysis-dependent at diagnosis (36% vs. 68%, p < 0.05). Despite the delay in diagnosis, there was a trend towards lower serum creatinine (392 vs. 591 micro M), lower relapse rate (0.04 versus 0.15 episodes/patient-year) and lower incidence of death/end-stage renal failure (27% vs. 53%) in the PMR(+) group. DISCUSSION: SVV is frequently misdiagnosed as PMR, especially in those patients with indolent disease, although this did not appear to adversely affect outcome. We recommend that all patients suspected of suffering from PMR undergo careful urinalysis to look for haematuria or proteinuria, and that a low threshold for ANCA testing is maintained.  相似文献   

9.
Todd D. Rozen MD  FAAN 《Headache》2010,50(9):1516-1519
Giant cell arteritis (GCA) should be considered in the differential diagnosis of any new onset headache occurring in individuals over the age of 50 years. Headache is the most common complaint in GCA patients but the clinical characteristics of the headache itself does not help in making a diagnosis as the headache can occur anywhere on the head, not just the temples, be mild to severe in intensity and be dull to throbbing in quality. As other things can cause new onset headache in older individuals, additional clinical symptoms or signs that may suggest GCA as a diagnosis would be useful to clinicians. Two cases are presented that suggests that new onset stabbing headache associated with a new daily persistent headache is a possible diagnostic sign for a diagnosis of GCA. Nothing in the literature to date has mentioned new onset stabbing headache as part of the presenting symptom complex for GCA.  相似文献   

10.
Aim: The purpose of this study was to clarify the experiences of difficulty that patients with cancer faced in the learning process of progressive muscle relaxation (PMR) and to consider which kind of PMR guidance should be given to weak patients with cancer. Methods: The participants were instructed to practise PMR for 2 weeks when they were in good condition. The interview was conducted 1 week and 2 weeks after the PMR intervention. The 21 participants who experienced difficulty were analyzed. The analysis was completed by using the content analysis method. Results: Fifteen patients practised PMR by themselves one‐to‐two times per day. However, six patients practised PMR three‐to‐four times per week when they were in good condition. The interview answers fell into two categories: (i) experiences of difficulty that patients encountered in the learning process; and (ii) experiences in which patients had no feeling of change though they had practised PMR. The former category included the subcategories of experiences in which patients felt difficulty with PMR, experiences in which patients felt uncomfortable symptoms after PMR, and experiences in which patients did not feel it was good timing to learn PMR. Conclusion: It is easy for disease to progress in patients with cancer and those with poor strength, so it is necessary to match the functional status of individual patients. It also is important to support patients in practising PMR whenever they have the desire, by respecting their will.  相似文献   

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