School of Medicine and Health Sciences Poster Presentations

Title

An unusual presentation of giant cell arteritis causing delay in diagnosis

Document Type

Poster

Keywords

Giant Cell Arteritis; Vasculitis; MRI/MRA; bilateral vision loss; rheumatology

Publication Date

Spring 2017

Abstract

TITLE: AN UNUSUAL PRESENTATION OF GIANT CELL ARTERITIS CAUSING DELAY IN DIAGNOSIS

Authors: Awan, Seemal F, George Washington University Hospital;

Isaac-Elder, Kurt, George Washington University School of Medicine;

McBride, Erica, George Washington University Hospital

Giant Cell Arteritis (GCA) is a large artery vasculitis, affecting the aorta and its branches, that typically presents with jaw claudication, headache, fever, and monocular vision changes. Rarely, patients may present with bilateral acute vision loss and thus GCA is often overlooked as the primary etiology leading to diagnostic delays and significant morbidity to patients. Here we present a case of GCA that initially presented with bilateral acute vision changes.

A 71-year-old woman with past medical history significant for hypertension, asthma, and COPD presented to an outside hospital with a low grade fever, headache, back pain, and transient bilateral vision changes. She was admitted to the outside hospital and initiated on empiric antibiotics for presumed Pyelonephritis in the setting of urinalysis positive for leukocyte esterase and CBC with a leukocytosis of 20k.

Our patient developed persistent bilateral vision loss on the third day of admission prompting a workup for acute thromboembolic stroke. Initial imaging with a non-contrast CT head and non-contrast MRI brain did not show any ischemic or hemorrhagic changes. The patient's vision continued to deteriorate resulting in complete loss of visual acuity and her persistent vision loss prompted transfer to our tertiary academic hospital for further evaluation.

On admission to our hospital, the patient’s physical exam revealed temporal muscle tenderness, jaw tenderness, and complete visual field deficits, labs were significant for WBC 20K, ESR 110, and CRP 359, and imaging studies including MRI face/neck/orbit with and without contrast showed “nonspecific non-mass like enhancement of the retro-orbital fat, muscles of mastication” indicating an underlying inflammatory process. Infectious workup did not yield an etiology for her continued low grade fever including Chest X-Ray, CBC, Urinalysis, Blood Cultures, etc. Ophthalmology and Neurology were also consulted. Given the elevated inflammatory markers, persistent visual deficits, as well as clinic picture, our patient was initiated on high dose steroids upon transfer to our hospital. Bilateral temporal artery biopsies were performed revealing “lymphohistiocytic inflammation involving adventitia and branching vessels consistent with temporal arteritis.” Unfortunately, despite the initiation of high dose steroids, there was no improvement in our patient’s visual acuity and she was later discharged to a rehab facility for further occupational therapy in the setting of her new visual deficits.

This case illustrates a rare presentation of GCA that caused a diagnostic delay resulting in significant morbidity for our patient. Although bilateral vision loss is a rare presentation of GCA, this particular constellation of symptoms should have prompted earlier consideration of GCA and thus earlier initiation of steroids. Magnetic resonance imaging/angiography (MRI/MRA) could potentially be considered as part of the diagnostic workup as preliminary studies have shown abnormal contrast enhancement in patients with abnormal contrast enhancement1. Considering GCA early in the diagnostic process in a patient presenting with bilateral vision loss is vital and MRI/MRA may be part of the diagnostic workup for GCA in the near future.

References

1 Gene, Hunder G. "Diagnosis of Giant Cell (temporal) Arteritis." Diagnosis of Giant Cell (temporal) Arteritis. UpToDate, 25 Oct. 2016. Web. 28 Feb. 2017.

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Poster to be presented at GW Annual Research Days 2017.

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An unusual presentation of giant cell arteritis causing delay in diagnosis

TITLE: AN UNUSUAL PRESENTATION OF GIANT CELL ARTERITIS CAUSING DELAY IN DIAGNOSIS

Authors: Awan, Seemal F, George Washington University Hospital;

Isaac-Elder, Kurt, George Washington University School of Medicine;

McBride, Erica, George Washington University Hospital

Giant Cell Arteritis (GCA) is a large artery vasculitis, affecting the aorta and its branches, that typically presents with jaw claudication, headache, fever, and monocular vision changes. Rarely, patients may present with bilateral acute vision loss and thus GCA is often overlooked as the primary etiology leading to diagnostic delays and significant morbidity to patients. Here we present a case of GCA that initially presented with bilateral acute vision changes.

A 71-year-old woman with past medical history significant for hypertension, asthma, and COPD presented to an outside hospital with a low grade fever, headache, back pain, and transient bilateral vision changes. She was admitted to the outside hospital and initiated on empiric antibiotics for presumed Pyelonephritis in the setting of urinalysis positive for leukocyte esterase and CBC with a leukocytosis of 20k.

Our patient developed persistent bilateral vision loss on the third day of admission prompting a workup for acute thromboembolic stroke. Initial imaging with a non-contrast CT head and non-contrast MRI brain did not show any ischemic or hemorrhagic changes. The patient's vision continued to deteriorate resulting in complete loss of visual acuity and her persistent vision loss prompted transfer to our tertiary academic hospital for further evaluation.

On admission to our hospital, the patient’s physical exam revealed temporal muscle tenderness, jaw tenderness, and complete visual field deficits, labs were significant for WBC 20K, ESR 110, and CRP 359, and imaging studies including MRI face/neck/orbit with and without contrast showed “nonspecific non-mass like enhancement of the retro-orbital fat, muscles of mastication” indicating an underlying inflammatory process. Infectious workup did not yield an etiology for her continued low grade fever including Chest X-Ray, CBC, Urinalysis, Blood Cultures, etc. Ophthalmology and Neurology were also consulted. Given the elevated inflammatory markers, persistent visual deficits, as well as clinic picture, our patient was initiated on high dose steroids upon transfer to our hospital. Bilateral temporal artery biopsies were performed revealing “lymphohistiocytic inflammation involving adventitia and branching vessels consistent with temporal arteritis.” Unfortunately, despite the initiation of high dose steroids, there was no improvement in our patient’s visual acuity and she was later discharged to a rehab facility for further occupational therapy in the setting of her new visual deficits.

This case illustrates a rare presentation of GCA that caused a diagnostic delay resulting in significant morbidity for our patient. Although bilateral vision loss is a rare presentation of GCA, this particular constellation of symptoms should have prompted earlier consideration of GCA and thus earlier initiation of steroids. Magnetic resonance imaging/angiography (MRI/MRA) could potentially be considered as part of the diagnostic workup as preliminary studies have shown abnormal contrast enhancement in patients with abnormal contrast enhancement1. Considering GCA early in the diagnostic process in a patient presenting with bilateral vision loss is vital and MRI/MRA may be part of the diagnostic workup for GCA in the near future.

References

1 Gene, Hunder G. "Diagnosis of Giant Cell (temporal) Arteritis." Diagnosis of Giant Cell (temporal) Arteritis. UpToDate, 25 Oct. 2016. Web. 28 Feb. 2017.