Giant cell arteritis with normal inflammatory markers: case report and review of the literature
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CASE BASED REVIEW
Giant cell arteritis with normal inflammatory markers: case report and review of the literature Patrícia Martins 1,2 & Vítor Teixeira 1,2,3 & Filipa Jorge Teixeira 4 & Mário Canastro 4 & Ana Palha 5 & João Eurico Fonseca 1,2 & Cristina Ponte 1,2 Received: 24 January 2020 / Revised: 13 April 2020 / Accepted: 16 April 2020 # International League of Associations for Rheumatology (ILAR) 2020
Abstract Giant cell arteritis (GCA) is a granulomatous vasculitis involving large- and middle-sized arteries, with a predilection for cranial arteries. In this paper, we aim to describe a case of a biopsy- and ultrasound-proven GCA, presenting with anterior ischemic optic neuropathy (AION) and normal CRP and ESR at diagnosis and during follow-up, and to review the literature on GCA cases with low inflammatory markers, with a particular focus on the presence of eye involvement. Both CRP and ESR are important in the diagnosis of GCA; occurrence of this disease with normal acute phase reactants is unusual but does not rule out the diagnosis. Headache and visual manifestations were the most common symptoms reported in these cases; therefore, patients with the clinical suspicion of GCA require immediate ultrasound and/or temporal artery biopsy for correct diagnosis and treatment initiation in order to avoid permanent visual loss. Keywords Giant cell arteritis . inflammatory markers
Introduction Giant cell arteritis (GCA) is a granulomatous vasculitis involving large- and middle-sized arteries, with a predilection for cranial arteries [1]. It is the most common primary vasculitis in Europe and North America in patients over 50 years of age, and it affects two to three times more frequently the female gender. The typical features of GCA are new onset of headache accompanied by constitutional symptoms, scalp * Patrícia Martins [email protected] 1
Serviço de Reumatologia e Doenças Ósseas Metabólicas, Hospital de Santa Maria, CHULN, Centro Académico de Medicina de Lisboa, Lisbon, Portugal
2
Unidade de Investigação em Reumatologia, Instituto de Medicina Molecular, Faculdade de Medicina, Universidade de Lisboa, Centro Académico de Medicina de Lisboa, Lisbon, Portugal
3
Serviço de Reumatologia, Hospital de Faro, Centro Hospitalar Universitário do Algarve, Faro, Portugal
4
Serviço de Oftalmologia, Hospital de Santa Maria, CHULN, Lisbon, Portugal
5
Serviço de Anatomia Patológica, Hospital de Santa Maria, CHULN, Lisbon, Portugal
tenderness, jaw claudication, visual disturbances, and increased inflammatory markers. The most common ophthalmic manifestation is anterior ischemic optic neuropathy (AION), which can lead to irreversible blindness. Prompt diagnosis and treatment are essential to prevent visual loss [2]. Ultrasound (US) of the temporal and axillary arteries, showing a non-compressible “halo sign,” and/or a biopsy of the temporal artery (TAB), presenting transmural inflammation associated with marked disruption of the internal elastic membrane, are the current recommended tests to diagnose patients in w
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