Head and neck giant cell arteritis: an autoimmune disease with many faces

Abstract

Conclusion: A high rate of infrequent presentations of giant cell arteritis were seen in the ENT department and should be anticipated as a differential diagnosis in every older patient with odynophagia with high CRP values without cause in thorough ENT examination.

Objective: To describe the clinical manifestation of head and neck giant cell arteritis and to derive a diagnostic pathway covering atypical cases.

Method: Single-center, retrospective analysis of cases with GCA in the head and neck region (HN-GCA) (2002–2012) to describe the clinical presentation and to derive a diagnostic pathway covering manifestations presenting to an ENT department.

Results: Sixty-five patients were newly diagnosed with HN-GCA in the department of otolaryngology, ophthalmology and neurology. The most frequent symptoms were loss of vision (83%) and new onset headache (63%). Eight patients (12%) presented with infrequent manifestations, predominantly in the department of otorhinolaryngology. The most common atypical presentation (50%) was odynophagia in conjunction with high CRP values misleading to an infectious cause and delaying diagnosis. A diagnostic pathway for GCA was derived based on the ACR classification criteria and the clinical findings.

Chinese abstract

结论: ENT科发现巨细胞性动脉炎非频繁出现的高发生率, 对每个年龄较大的患有高CRP值食管炎患者, 应该是可预见的鉴别诊断, 无需进行彻底的ENT检查。

目的: 描述头颈巨细胞性动脉炎的临床表现, 并得出非典型病例的诊断途径。

方法: 对头颈部 (HN-GCA) 患有GCA的病例 (2002-2012) 进行单中心回顾性分析, 以描述临床表现, 并推导出涵盖提供给ENT科的病症表现的诊断途径。

结果: 耳鼻喉科、眼科和神经病科新诊断出65例HN-GCA病人。最常见的症状是视力丧失 (83%) 和新发头痛 (63%) 。 8例 (12%) 表现出罕见的病症, 主要发生在耳鼻喉科。最常见的非典型病症 (50%) 是食管炎伴有高CRP值, 误导为传染病并延迟诊断。基于ACR分类标准和临床发现, 得出了GCA的诊断途径。

Keywords:

• Giant cell arteritis
• head and neck
• atypical manifestation
• amaurosis
• diagnostic pathway

Acknowledgements

The authors gratefully acknowledge Dr. Gregor Babaryka from the Institute of Pathology, Technical University of Munich, for providing with image material of section of temporal artery biopsy.

Disclosure statement

The authors declare no conflict of interest.

Ethics approval and consent to participate

Only routine clinical data were retrospectively collected from patients’ charts and pseudonymously analyzed and therefore no formal ethical approval and no consent to participate was obtained.