Department of Internal Medicine and RECIF, Amiens University Hospital, France.
Drugs Aging. 2011 Aug 1;28(8):651-66. doi: 10.2165/11592500-000000000-00000.
Giant cell arteritis (GCA) is an inflammatory vasculopathy that involves large- and medium-sized arteries and can cause vision loss, stroke and aneurysms. GCA occurs in people aged >50 years and is more common in women. A higher incidence of the disease is observed in populations from Northern European countries. Polymyalgia rheumatica (PMR) is a periarticular inflammatory process manifesting as pain and stiffness in the neck, shoulders and pelvic girdle. PMR shares the same pattern of age and sex distribution as GCA. The pathophysiology of PMR and GCA is not completely understood, but the two conditions may be related and often occur concurrently. A delay in the diagnosis should be avoided because of the risk of vascular ischaemic complications due to GCA. The diagnosis should be considered in patients aged >50 years presenting with symptoms such as new headache, visual disturbances, jaw claudication or symptoms of PMR. GCA can also present as a systemic inflammatory syndrome with fever of unknown origin. Marked elevation of acute-phase reactants, recognizable in higher erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) levels, is often seen in both PMR and GCA. However, some patients can present with a normal ESR. Confirmation of the diagnosis of GCA by temporal artery biopsy is important because clinical findings and laboratory tests are not specific, and because a diagnosis of GCA commits patients to long-term treatment with corticosteroids. The role of imaging techniques for the diagnosis of GCA remains unclear, but these modalities can be helpful in assessing the extent of vascular involvement, especially when extra-cranial disease is present. In PMR, subdeltoid and subacromial bursitis can be identified by imaging techniques, especially ultrasound or MRI. The clinical manifestations of GCA and PMR respond dramatically within 12-48 hours of starting corticosteroid treatment. The initial corticosteroid dosage commonly used in GCA is oral prednisone 40-60 mg/day, and for patients with PMR a dosage of 15-20 mg/day is often sufficient. A prolonged course of treatment is necessary, and corticosteroids are gradually tapered, guided by regular clinical evaluation and ESR (and/or CRP) measurement. Methotrexate is the best studied corticosteroid-sparing agent in GCA, and may be useful for patients with frequent disease relapses and/or corticosteroid-related toxicity. Retrospective studies favour aspirin (acetylsalicylic acid) as an effective adjuvant treatment for reducing the ischaemic complications of GCA. The long-term course of corticosteroid therapy frequently exposes elderly patients with PMR/GCA to various adverse effects, which can be attenuated with appropriate prophylactic measures. Co-morbid diseases and polypharmacy can pose particular challenges in the geriatric population. In general, the life expectancy of patients with GCA does not appear to be shortened, whereas the morbidity associated with the disease and its treatment is well recognized.
巨细胞动脉炎(GCA)是一种累及大动脉和中动脉的炎症性血管病,可导致视力丧失、中风和动脉瘤。GCA 发生于年龄>50 岁的人群,女性更为常见。北欧国家的人群发病率更高。风湿性多肌痛(PMR)是一种关节周围炎症性疾病,表现为颈部、肩部和骨盆带疼痛和僵硬。PMR 的发病年龄和性别分布与 GCA 相同。PMR 和 GCA 的病理生理学尚未完全阐明,但这两种疾病可能相关,并且常同时发生。由于 GCA 导致的血管缺血性并发症风险,应避免延迟诊断。对于出现新头痛、视力障碍、下颌跛行或 PMR 症状的年龄>50 岁的患者,应考虑诊断 GCA。GCA 也可表现为原因不明的发热性全身炎症综合征。PMR 和 GCA 中常可见明显的急性期反应物升高,表现为红细胞沉降率(ESR)和 C 反应蛋白(CRP)水平升高。然而,一些患者的 ESR 可能正常。颞动脉活检对 GCA 的诊断很重要,因为临床发现和实验室检查不具有特异性,并且 GCA 的诊断需要患者长期接受皮质类固醇治疗。影像学技术在 GCA 诊断中的作用尚不清楚,但这些方法有助于评估血管受累的程度,尤其是当存在颅外疾病时。在 PMR 中,肩胛下和肩峰下滑囊炎可通过影像学技术(尤其是超声或 MRI)识别。GCA 和 PMR 的临床表现在开始皮质类固醇治疗后 12-48 小时内会显著改善。GCA 常用的初始皮质类固醇剂量为口服泼尼松 40-60mg/天,而 PMR 患者的剂量通常为 15-20mg/天。需要长期治疗,根据定期临床评估和 ESR(和/或 CRP)测量逐渐减少皮质类固醇剂量。甲氨蝶呤是 GCA 中研究最多的皮质类固醇维持治疗药物,对于频繁复发和/或皮质类固醇相关毒性的患者可能有用。回顾性研究支持阿司匹林(乙酰水杨酸)作为降低 GCA 缺血性并发症的有效辅助治疗药物。皮质类固醇长期治疗常使 PMR/GCA 的老年患者面临各种不良反应,适当的预防措施可减轻这些不良反应。合并疾病和多种药物治疗给老年人群带来了特殊挑战。一般来说,GCA 患者的预期寿命似乎没有缩短,而疾病及其治疗相关的发病率是公认的。