Extracranial as Silent Giant Cell Arteritis: Case Report

Abstract

Giant cell arteritis (GCA) is a granulomatous vasculitis of medium and large arteries, frequently presenting with typical cranial symptoms, but sometimes nonspecific extracranial involvements are dominant. Diagnosis of this “occult” or “extracranial” GCA as a medical emergency is crucial to preventing irreversible complications [1, 2]. GCA vasculitis involves largeto medium-sized arteries, especially the aorta and proximal branches. Its classic presentations as a cranial arteritis comprise temporal headache, visual disturbance, and jaw claudication [2, 3]. In recent years, interest in attaining knowledge about cases with non-classic manifestations and involvement of extracranial arteries has increased [1]. Because of the nonspecific manifestations of extracranial GCA, it seems that actual incidence prevalence, which is reported as only 1.632.8 per 100,000 people, has been underestimated [1]. Common symptoms of extracranial GCA include fever, anorexia, malaise, weight loss, polymyalgia, and muscle weakness. Some vascular manifestations are more specific but less common, such as Raynaud’s phenomenon, digital ischemia, decreased pulse, limb claudication, arterial bruits, and signs of cerebral ischemia. In comparison with cranial GCA, extracranial GCA is more prevalent in women, and the onset of disease occurs at younger ages. Moreover, there is a longer delay in diagnosis. Therefore, in patients over 50 years of age with constitutional raised inflammatory markers, a diagnosis of extracranial GCA must be considered [1]. High risk for developing aneurysms and dissection of the aorta (especially thoracic segment) necessitates prompt diagnosis and treatment [3]. Because of the non-specific signs, symptoms, and blood tests and the difficulties in biopsying affected arteries, extracranial GCA is often diagnosed based on imaging. Acute aortic pathology as the first clinical presentation with a high mortality rate (44-80%) raises suspicion of previously established extracranial involvement. The gold standard for diagnosing extracranial GCA used to be conventional angiography, but that has been replaced with non-invasive methods [3]. Despite the lack of a specific laboratory marker, acute phase reactants (platelets, erythrocyte sedimentation rate, and/or C reactive protein) are raised in most patients. Normal values, however, in the presence of strong clinical suspicion would not rule out the diagnosis [3]. In patients with typical cranial presentation, duplex ultrasonography of the temporal artery is a proper diagnostic modality. In addition, diagnosing patients with predominant extracranial manifestations and atypical ones may be aided by other imaging techniques, such as CT (computed tomography), MRI (magnetic resonance imaging), CT-angiography, and PET (positron emission tomography). The widespread use of these new imaging techniques may lead to the identification of clinically silent large vessel involvement, and this will probably raise the rate of incidence [3]. In diagnosing GCA, a specialist clinical evaluation, which can be based on signs, symptoms, and laboratory Case Report Open Access