A case of vision loss in a patient with Giant Cell Arteritis
Henry Ford Health System
Background: Giant Cell Arteritis (GCA) is the most common large vessel vasculitis (1) and carries the potential for life-changing morbidity manifested as permanent vision loss or other neurological de..more »
Background: Giant Cell Arteritis (GCA) is the most common large vessel vasculitis (1) and carries the potential for life-changing morbidity manifested as permanent vision loss or other neurological deficit. Despite these well understood complications, literature suggests that a significant delay to diagnosis in GCA exists. (2) Owing to the gradual and non-specific symptoms associated with GCA, patients are likely to present in the ambulatory setting. This case report aims to provide an example of a delay to diagnosis in GCA and improve awareness of the diagnostic challenges facing primary healthcare providers.
Case Description: An 80 year old Caucasian female presented to her PCP with a 1 week history of aching left sided neck pain with radiation to posterior scalp. She denied any associated symptoms. Medical history included Coronary Artery Disease and Right Supraspinatus Tear. Neck pain was initially diagnosed as cervical spondylosis and the patient was discharged with physical therapy referral. Over the following weeks the patient presented twice to her PCP and once to the Emergency Department (ED) with symptoms including scalp pain, fatigue, persistent headache, pain in her mouth and weight loss. Work-up during this time included routine labs and MRI Brain and C-spine which did not reveal a diagnosis. 8 weeks following the onset of symptoms the patient presented to the ED with partial vision loss in her right eye. ESR was found to be elevated at 47mm/hr. She was started on Prednisone 60mg PO daily and underwent Temporal Artery Biopsy (TAB) which confirmed the diagnosis of Giant Cell Arteritis. Despite oral steroids she developed vision loss in the contralateral eye and was admitted to hospital to receive IV corticosteroids. She was subsequently started on Tocilizumab (Actemra) with tapering of corticosteroids. 4 months following her initial vision loss she reported no improvement in vision despite adherence to therapy. 6 months after the diagnosis of GCA she was admitted for severe sepsis and lower GI bleeding secondary to terminal ileitis. She had poor functional recovery following discharge from hospital and died under hospice care 7 months later.
Discussion: A recent meta-analysis estimates the mean time from presentation to diagnosis of GCA at 9 weeks.(2) This case demonstrates a similar pattern and highlights the challenge and importance of achieving a prompt diagnosis. Existing literature suggests that few of the classic symptoms and physical signs are predictive of biopsy-confirmed GCA.(3) Furthermore, none of these history and physical points are reliable to rule out GCA. However, Erythrocyte Sedimentation Rate (ESR) has demonstrated utility in ruling out GCA with reported sensitivity ranging from 85-96% for any ESR elevation and Negative Likelihood Ratio of 0.2 for normal ESR (3,4). The American College of Rheumatology published Classification Criteria for GCA in 1990, however comprehensive management guidelines are still under development. Existing European and British guidelines endorse maintaining a high-degree of clinical suspicion to prompt urgent referral for TAB and specialist evaluation. (5, 6). To improve clinical outcomes through rapid diagnosis, studies of fast-track referral pathways have demonstrated reduced rates of permanent-vision loss compared to conventional care (7). Nonetheless, increased primary care awareness is necessary for consideration of this less common but potentially catastrophic diagnosis.
Henry Ford Hospital
Resident PGY 3
Henry Ford Health System