432. Ischemic Optic Neuropathy Secondary to Varicella Zoster Vasculitis Mimicking Giant Cell Arteritis
Creators: name: Rodriguez-Pla1, Alicia
Publisher: name: Zenodo
Year: 2022
Subjects: Vasculitis
ANCA
Abstracts
Giant Cell Arthritis
MPA
GPA
EGPA
Takayasu
IgA vasculitis

description: <strong>Presentation of Case: </strong>An 82-year-old woman presented with sudden onset of a blind spot in her left eye, which she first noticed while watching TV 3 weeks prior and had remained unchanged. She had bilateral optic disc edema and peripapillary haemorrhages, raising concerns for giant cell arteritis (GCA). Her past medical history was relevant for multifocal atrial tachycardia, diverticulitis with sigmoid perforation requiring colostomy, remote history of melanoma, stage 3 chronic kidney disease, lower extremity edema, and viral epiglottis complicated by sepsis a few months prior to her current presentation. Her family history was remarkable for cancer.She had not had any recent onset of a new type of headache, eye pain, fevers, night sweats, weight loss, jaw pain or claudication, scalp tenderness, or pain, weakness or stiffness in shoulders or hip girdles. Rest of review of systems was negative.The patient’s vitals were within normal limits. Her physical exam was unremarkable but for ostomy and 1+ pitting in lower extremities. <strong>Diagnostic Testing: </strong>Her labs were within normal limits but for mild anemia with hemoglobin: 11.3 g/dL, erythrocyte sedimentation rate (ESR): 42 mm/hr, BUN: 25.3 mg/dL, creatinine: 1.05 mg/dL, eGFR: 50 mL/min, C-reactive protein (CRP): 16.1 mg/L. Immunological tests were all negative.An electrocardiogram, chest X-ray, chest computerized (CT) angiogram, brain magnetic resonance (MR) imaging (MRI), and neck MR angiogram (MRA) were unrevealing. An MRI of the orbits revealed prominent vessels in the right greater than the left temporal scalp, suggestive of temporal arteritis. A brain MRA revealed a long segment mild-to-moderate stenosis of the proximal basilar artery, and mild asymmetric narrowing of the left middle cerebral artery M2 branches (Figure 1A and 1B). Bilateral temporal artery biopsies revealed arteriosclerosis with local calcifications but no inflammation. <strong>Differential &amp; Final Diagnosis: </strong>The presentation in an elderly patient of visual loss and elevated inflammatory markers was concerning for GCA. However, the differential diagnosis was broad and includes non-arteritis ischemic optic neuritis, tumor, demyelinating disease, elevated intracranial pressure due to thrombosis of the cerebral vein and/or sinuses, infectious, other vasculitides or paraneoplastic.Given the suspicion for biopsy-negative GCA with visual involvement, the patient received 1 gram of intravenous (IV) methylprednisolone daily for 3 days and was transitioned to oral prednisone 60 mg orally daily with plan to discuss about tocilizumab initiation during her follow up.A week later, she was evaluated by neuro-ophthalmology. Funduscopic examination demonstrated right optic disc edema and left pallid optic disc with bilateral peripapillary haemorrhages. Despite high dose glucocorticoid treatment, the CRP and ESR remained elevated. A lumbar puncture revealed normal opening pressure, without elevation in CSF protein, but the CSF polymerase chain reaction was positive for varicella-zoster virus (VZV). The patient had a prior history of chickenpox in the early teenage years and zoster as an adult 14 years prior, and she had received the shingles vaccine. The final diagnosis was bilateral arteritic ischemic optic neuropathy secondary to VZV infection. <strong>Discussion of Management: </strong>She was treated with IV acyclovir for 14 days through a PICC line and her prednisone was rapidly tapered off. Her inflammatory markers quickly normalized. A brain MRA repeated 2 months later showed improvement in the basilar artery stenosis (Figure 1C). <strong>Conclusions: </strong>Differentiating GCA from its many mimickers remains a challenge in the clinical practice. In patients with negative temporal artery biopsy, the persistence of elevated inflammatory markers despite high dose steroids, should preclude additional work up for the search of an alternative diagnosis. <strong>Disclosures: </strong>The authors have nothing to disclose