de Boysson Hubert, Lambert Marc, Liozon Eric, Boutemy Jonathan, Maigné Gwénola, Ollivier Yann, Ly Kim, Manrique Alain, Bienvenu Boris, Aouba Achille
aDepartment of Internal Medicine, Caen University Hospital bDepartment of Internal Medicine, Lille University Hospital cDepartment of Internal Medicine, Limoges University Hospital dDepartment of Nuclear Medicine, Caen University Hospital eNormandy University, Caen, France.
Medicine (Baltimore). 2016 Jun;95(26):e3818. doi: 10.1097/MD.0000000000003818.
Diagnosis of giant-cell arteritis (GCA) is challenging in the absence of cardinal cranial symptoms/signs. We aimed to describe the clinical presentation, diagnostic process, and disease course of GCA patients without cranial symptoms, and to compare them to those of patients with typical cranial presentation. In this retrospective multicenter study, we enrolled patients with GCA who satisfied at least 3 of the 5 American College of Rheumatology criteria for GCA, or 2 criteria associated with contributory vascular biopsy other than temporal artery biopsy or with demonstration of large-vessel involvement; underwent iconographic evaluation of large arterial vessels (aortic CT scan or a positron emission tomography with F-fluorodeoxyglucose combined with computed tomography (FDG-PET/CT) scan or cardiac echography combined with a large-vessel Doppler) at diagnosis. We divided the cohort into 2 groups, distinguishing between patients without cranial symptoms/signs (i.e., headaches, clinical temporal artery anomaly, jaw claudication, ophthalmologic symptoms) and those with cranial symptoms/signs. In the entire cohort of 143 patients, all of whom underwent vascular biopsy and vascular imaging, we detected 31 (22%) patients with no cranial symptoms/signs. In the latter, diagnosis was biopsy proven in an arterial sample in 23 cases (74% of patients, on a temporal site in 20 cases and on an extratemporal site in 3). One-third of these 31 patients displayed extracranial symptoms/signs whereas the remaining two-thirds presented only with constitutional symptoms and/or inflammatory laboratory test results. Compared to the 112 patients with cardinal cranial clinical symptoms/signs, patients without cranial manifestations displayed lower levels of inflammatory laboratory parameters (C-reactive level: 68 [9-250] mg/L vs 120 [3-120] mg/L; P < 0.01), highest rate of aorta and aortic branch involvement identified (19/31 (61%) vs 42/112 (38%); P = 0.02) and also a lower rate of disease relapse (12/31 (39%) vs 67/112 (60%); P = 0.04). Our results suggest that patients without cranial symptoms/signs are prone to lower inflammatory laboratory parameters, fewer relapses, and more large-vessel involvement than those displaying cardinal cranial manifestations. Further studies are therefore required in order to determine whether these 2 subgroups of patients have a different prognosis, and therefore warrant different therapeutic and monitoring regimens.
在缺乏典型颅脑症状/体征的情况下，巨细胞动脉炎（GCA）的诊断具有挑战性。我们旨在描述无颅脑症状的GCA患者的临床表现、诊断过程及疾病进程，并将其与有典型颅脑表现的患者进行比较。在这项回顾性多中心研究中，我们纳入了符合美国风湿病学会GCA 5项标准中至少3项，或符合除颞动脉活检外其他有助于诊断的血管活检标准或显示大血管受累标准的GCA患者；在诊断时对大动脉血管进行影像学评估（主动脉CT扫描、氟脱氧葡萄糖正电子发射断层扫描联合计算机断层扫描（FDG-PET/CT）或心脏超声联合大血管多普勒检查）。我们将该队列分为两组，区分无颅脑症状/体征（即头痛、临床颞动脉异常、颌部间歇性运动障碍、眼科症状）的患者和有颅脑症状/体征的患者。在全部143例均接受了血管活检和血管成像的患者队列中，我们检测到31例（22%）无颅脑症状/体征。在后者中，23例（74%的患者）经动脉样本活检确诊，其中20例为颞部活检，3例为颞外部位活检。这31例患者中有三分之一表现出颅外症状/体征，而其余三分之二则仅表现为全身症状和/或炎症实验室检查结果。与112例有典型颅脑临床症状/体征的患者相比，无颅脑表现的患者炎症实验室指标水平较低（C反应蛋白水平：68 [9-250]mg/L对120 [3-120]mg/L；P<0.01），主动脉及主动脉分支受累率最高（19/31（61%）对42/112（38%）；P = 0.02），疾病复发率也较低（12/31（39%）对67/112（60%）；P = 0.04）。我们的结果表明，与有典型颅脑表现的患者相比，无颅脑症状/体征的患者炎症实验室指标较低，复发较少，大血管受累较多。因此，需要进一步研究以确定这两组患者的预后是否不同，以及是否需要不同的治疗和监测方案。
