Gow James, Roofeh David
Rutgers-Robert Wood Johnson Medical School, New Brunswick, NJ, 08854, USA.
Department of Molecular Biology, Princeton University, Princeton, NJ, 08540, USA.
BMC Rheumatol. 2025 May 14;9(1):53. doi: 10.1186/s41927-025-00505-y.
Giant cell arteritis (GCA) is a large vessel vasculitis characterized by granulomatous inflammation classically affecting the carotid artery branches. GCA most often presents with one or more classic clinical features which include headache, jaw claudication, temporal scalp tenderness, and polymyalgia rheumatica. In a minority of cases, GCA can adopt an "occult" presentation (i.e., failure to thrive in the setting of unexplained inflammation) where vascular manifestations affect vascular beds, such as lingual ulceration, not amenable to biopsy. While the diagnosis of GCA is often supported by temporal artery biopsy or imaging studies, such as temporal artery ultrasound or magnetic resonance angiography, these techniques are known to have limited sensitivity. As a result, there is the potential for GCA to be misdiagnosed where it presents both in the absence of classic clinical manifestations and without clear diagnostic evidence by imaging or histopathology.
A 78-year-old male presented to rheumatology on the inpatient consult service with unexplained headaches, failure to thrive, and persisting elevated acute phase reactants. He was admitted for unexplained fevers three times in as many months, with an unrevealing infectious and malignancy workup. His past medical history was remarkable for a shallow right lateral tongue ulcer that was non-healing despite weeks of acyclovir treatment. Two bitemporal artery ultrasounds did not suggest features of GCA and subsequent temporal artery biopsies failed to show healing or active arteritis. The patient was started on empiric corticosteroids tapered to discontinue over six months in conjunction with tocilizumab. He had rapid normalization of inflammatory markers (prior to tocilizumab initiation), anemia of chronic inflammation, and correction of his serum Na + without need for ongoing fluid restriction. Clinically, his headaches and unexplained weight loss improved and his serial exams showed complete resolution of his tongue ulcer, suspected to be end-organ damage from GCA.
Although the hospitalist service suspected GCA in this elderly patient with headaches, failure to thrive, recurrent fever of unknown origin, and elevated inflammatory markers, they were deterred from this diagnosis by repeat negative bitemporal artery ultrasounds and negative biopsies. This case demonstrates the need to survey for atypical vascular beds of GCA involvement, even in the presence of negative imaging and biopsy results.
巨细胞动脉炎（GCA）是一种大血管血管炎，其特征为肉芽肿性炎症，典型情况下会累及颈动脉分支。GCA最常表现为一种或多种典型临床特征，包括头痛、颌部间歇性运动障碍、颞部头皮压痛和风湿性多肌痛。在少数情况下，GCA可呈现“隐匿性”表现（即，在不明原因炎症的情况下生长发育不良），此时血管表现累及血管床，如舌部溃疡，而无法进行活检。虽然GCA的诊断通常通过颞动脉活检或影像学检查（如颞动脉超声或磁共振血管造影）来支持，但已知这些技术的敏感性有限。因此，在GCA既无典型临床表现，又无影像学或组织病理学明确诊断证据的情况下，有可能被误诊。
一名78岁男性因不明原因头痛、生长发育不良和急性期反应物持续升高，入住住院医师会诊服务的风湿科。他在三个月内因不明原因发热入院三次，感染和恶性肿瘤检查均无异常发现。他既往有右侧舌外侧浅表溃疡病史，尽管接受了数周的阿昔洛韦治疗仍未愈合。两次颞动脉超声检查未提示GCA特征，随后的颞动脉活检未显示愈合或活动性动脉炎。患者开始接受经验性糖皮质激素治疗，并联合托珠单抗在六个月内逐渐减量停药。他的炎症标志物迅速恢复正常（在开始使用托珠单抗之前），慢性炎症性贫血得到改善，血清钠+恢复正常，无需持续限制液体摄入。临床上，他的头痛和不明原因体重减轻有所改善，连续检查显示舌部溃疡完全愈合，怀疑是GCA导致的终末器官损伤。
尽管住院医师服务怀疑这名患有头痛、生长发育不良、不明原因反复发热和炎症标志物升高的老年患者患有GCA，但反复的双侧颞动脉超声检查阴性和活检阴性使他们排除了这一诊断。该病例表明，即使影像学和活检结果为阴性，也需要检查GCA累及的非典型血管床。
