Vita-Salute San Raffaele University, Milan, Italy; Department of Medicine and Clinical Immunology, IRCCS San Raffaele Scientific Institute, Milan, Italy.
Vita-Salute San Raffaele University, Milan, Italy; Department of Medicine and Clinical Immunology, IRCCS San Raffaele Scientific Institute, Milan, Italy.
Autoimmun Rev. 2015 Apr;14(4):352-7. doi: 10.1016/j.autrev.2014.12.005. Epub 2014 Dec 17.
Giant cell arteritis (GCA) is a systemic vasculitis typically affecting temporal arteries. In at least 15% of cases, GCA also features inflammation of the aorta and its primary branches. Large-vessel inflammation restricted to proximal limb arteries in the absence of temporal and aortic involvement (Limb Restricted, LR) is rare and not well described in literature. Hence, we aim to characterize this neglected clinical entity.
We describe a series of three cases of LR-GCA. All patients were older than 50 years, had increased erythrocyte sedimentation rate (ESR), normal cholesterol and triglycerides serum levels, negative temporal artery biopsy, suggestive F-18 fluorodeoxyglucose positron emission tomography (FDG-PET) findings, and responded to immunosuppressive therapy. We also reviewed all published cases of LR-GCA (76 cases), for a total of 79 patients.
Limb claudication was reported in 87% of the patients, and cranial symptoms and polymyalgia rheumatica in 20%. Constitutional symptoms were never reported. Median ESR levels were 66.5mm/1h. Upper and lower limb arteries were involved in 86% and 9% of the patients respectively, and the remaining 5% had simultaneous upper and lower limb vessel involvement. Conventional angiography was performed in 63% of the cases, color-doppler ultrasound in 20%, FDG-PET in 14%, and computed tomography angiography in 3%.
If temporal biopsy and aortic imaging are negative for GCA in patients older than 50 years with bilateral limb claudication, elevated ESR, and suggestive vascular radiological findings, LR-GCA should be suspected. Upper limb arteries are more frequently involved. Since constitutional symptoms are typically absent in LR-GCA, differential diagnosis with atherosclerotic plaques may be challenging.
巨细胞动脉炎（GCA）是一种系统性血管炎，通常影响颞动脉。在至少 15%的病例中，GCA 还伴有主动脉及其主要分支的炎症。在没有颞动脉和主动脉受累的情况下，仅局限于肢体近端动脉的大血管炎症（肢体局限型，LR）很少见，文献中也没有很好的描述。因此，我们旨在描述这一被忽视的临床实体。
我们描述了三例 LR-GCA 患者。所有患者均年龄>50 岁，红细胞沉降率（ESR）升高，胆固醇和甘油三酯血清水平正常，颞动脉活检阴性，氟-18 脱氧葡萄糖正电子发射断层扫描（FDG-PET）结果提示，对免疫抑制治疗有反应。我们还回顾了所有已发表的 LR-GCA 病例（76 例），共 79 例患者。
87%的患者报告有肢体跛行，20%的患者有颅神经症状和风湿性多肌痛。从未报告过全身症状。中位 ESR 水平为 66.5mm/1h。上、下肢动脉受累分别占 86%和 9%，其余 5%同时累及上、下肢血管。63%的病例进行了常规血管造影，20%进行了彩色多普勒超声检查，14%进行了 FDG-PET 检查，3%进行了计算机断层血管造影检查。
如果 50 岁以上的患者双侧肢体跛行、ESR 升高且血管影像学检查提示可疑，且颞动脉活检和主动脉成像均无 GCA 表现，应怀疑 LR-GCA。上肢动脉更常受累。由于 LR-GCA 通常无全身症状，与动脉粥样硬化斑块的鉴别诊断可能具有挑战性。
