巨细胞动脉炎(GCA)的不典型表现:78岁男性颞动脉活检阴性的体质症状和舌溃疡。

IF 2.1 Q3 RHEUMATOLOGY
James Gow, David Roofeh
{"title":"巨细胞动脉炎(GCA)的不典型表现:78岁男性颞动脉活检阴性的体质症状和舌溃疡。","authors":"James Gow, David Roofeh","doi":"10.1186/s41927-025-00505-y","DOIUrl":null,"url":null,"abstract":"<p><strong>Background: </strong>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.</p><p><strong>Case presentation: </strong>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.</p><p><strong>Conclusions: </strong>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.</p>","PeriodicalId":9150,"journal":{"name":"BMC Rheumatology","volume":"9 1","pages":"53"},"PeriodicalIF":2.1000,"publicationDate":"2025-05-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12080050/pdf/","citationCount":"0","resultStr":"{\"title\":\"An atypical manifestation of Giant cell arteritis (GCA): constitutional symptoms & lingual ulcer in a 78-Year-Old male with negative temporal artery biopsies.\",\"authors\":\"James Gow, David Roofeh\",\"doi\":\"10.1186/s41927-025-00505-y\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><strong>Background: </strong>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.</p><p><strong>Case presentation: </strong>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.</p><p><strong>Conclusions: </strong>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.</p>\",\"PeriodicalId\":9150,\"journal\":{\"name\":\"BMC Rheumatology\",\"volume\":\"9 1\",\"pages\":\"53\"},\"PeriodicalIF\":2.1000,\"publicationDate\":\"2025-05-14\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12080050/pdf/\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"BMC Rheumatology\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.1186/s41927-025-00505-y\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q3\",\"JCRName\":\"RHEUMATOLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"BMC Rheumatology","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.1186/s41927-025-00505-y","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q3","JCRName":"RHEUMATOLOGY","Score":null,"Total":0}
引用次数: 0

摘要

背景:巨细胞动脉炎(GCA)是一种以肉芽肿性炎症为特征的大血管炎,典型地影响颈动脉分支。GCA最常表现为一种或多种典型临床特征,包括头痛、下颌跛行、颞部头皮压痛和风湿性多肌痛。在少数病例中,GCA可表现为“隐匿性”表现(即,在不明原因的炎症背景下不能生长),其中血管表现影响血管床,如舌溃疡,无法进行活检。虽然GCA的诊断通常由颞动脉活检或成像研究支持,如颞动脉超声或磁共振血管造影,但已知这些技术的灵敏度有限。因此,在没有典型临床表现和没有明确的影像学或组织病理学诊断证据的情况下,GCA有可能被误诊。病例介绍:一名78岁男性,因不明原因的头痛、生长衰竭和持续急性期反应物升高而就诊于风湿病住院会诊。他在三个月内三次因不明原因的发烧而入院,并进行了未透露的感染和恶性检查。他过去的病史是显著的浅舌侧溃疡,尽管几周的阿昔洛韦治疗仍未愈合。两次双颞动脉超声检查未发现GCA的特征,随后的颞动脉活检未能显示愈合或活跃的动脉炎。患者开始使用经年性皮质类固醇,逐渐减少到6个月后停止使用托珠单抗。他的炎症标志物迅速正常化(在托珠单抗开始前),慢性炎症贫血,血清Na +得到纠正,无需持续限制液体。临床上,他的头痛和不明原因的体重减轻有所改善,他的系列检查显示他的舌头溃疡完全消退,怀疑是GCA引起的终末器官损害。结论:虽然医院怀疑这名老年患者有头痛、身体发育不佳、反复出现不明原因发热和炎症标志物升高等症状,但反复双颞动脉超声检查和活检均呈阴性,使他们无法做出这种诊断。本病例表明,即使在影像学和活检结果阴性的情况下,也需要对GCA累及的非典型血管床进行检查。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
An atypical manifestation of Giant cell arteritis (GCA): constitutional symptoms & lingual ulcer in a 78-Year-Old male with negative temporal artery biopsies.

Background: 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.

Case presentation: 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.

Conclusions: 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.

求助全文
通过发布文献求助,成功后即可免费获取论文全文。 去求助
来源期刊
BMC Rheumatology
BMC Rheumatology Medicine-Rheumatology
CiteScore
3.80
自引率
0.00%
发文量
73
审稿时长
15 weeks
×
引用
GB/T 7714-2015
复制
MLA
复制
APA
复制
导出至
BibTeX EndNote RefMan NoteFirst NoteExpress
×
提示
您的信息不完整,为了账户安全,请先补充。
现在去补充
×
提示
您因"违规操作"
具体请查看互助需知
我知道了
×
提示
确定
请完成安全验证×
copy
已复制链接
快去分享给好友吧!
我知道了
右上角分享
点击右上角分享
0
联系我们:info@booksci.cn Book学术提供免费学术资源搜索服务,方便国内外学者检索中英文文献。致力于提供最便捷和优质的服务体验。 Copyright © 2023 布克学术 All rights reserved.
京ICP备2023020795号-1
ghs 京公网安备 11010802042870号
Book学术文献互助
Book学术文献互助群
群 号:481959085
Book学术官方微信