Case 11 - Neuro-ophthalmology, orbit, and inflammation

Sudden Vision Loss with Headache and Shoulder Pain

70-year-old woman - Sudden painless monocular vision loss - Headaches + shoulder pain x months

Illustrated eye for pathology case review

Case 11Giant Cell Arteritis / AION

Clinical Presentation

A 70-year-old woman suffered sudden but painless vision loss in one eye. She has also experienced headaches and shoulder pain for the past several months. The affected eye is shown; her other eye remains normal.

Learner Questions

What questions would you include when taking the history?
Describe the ophthalmoscopic findings.
What is the differential diagnosis?
What should the diagnostic workup include?
What are your strategies for short- and long-term management?

Answer Framework

History

Jaw claudication (most specific - ~65% sensitive), scalp tenderness, temporal artery tenderness/nodularity, PMR symptoms (proximal girdle stiffness worse in AM), prior transient monocular VL, constitutional symptoms (fever, weight loss), diplopia, prior elevated ESR/CRP.

Workup

START STEROIDS IMMEDIATELY - do not wait for biopsy results. ESR (Westergren, often >100) + CRP (more sensitive - elevated in nearly all active GCA). CBC (thrombocytosis, anemia). Temporal artery biopsy (>=2 cm, within 1-2 weeks after starting steroids - histology remains positive). Color duplex US (halo sign around temporal arteries). PET-CT for large-vessel involvement.

Findings

Pale, swollen optic disc with CHALKY-WHITE PALLOR (more profound than NAION) and blurred margins. Possible segmental/altitudinal disc edema. Flame hemorrhages at disc margin. Chalky-white disc = hallmark of arteritic AION. Concurrent cilioretinal artery occlusion possible.

Management

Acute: IV methylprednisolone 250-500 mg q6h if vision already lost (may protect fellow eye). Oral prednisone 1 mg/kg/day if vision intact. Long-term: slow taper over 1-2 years guided by ESR/CRP. Tocilizumab (IL-6 inhibitor - GiACTA trial) = steroid-sparer, reduces relapse + cumulative steroid dose. Low-dose aspirin 81 mg. Annual CXR for aortic aneurysm.

DDx

Arteritic AION (GCA) - top here: chalky disc + systemic symptoms + elevated ESR/CRP. Non-arteritic AION (NAION) - small disc-at-risk, vasculopathic risk factors (HTN/DM/sleep apnea), normal inflammatory markers, altitudinal field defect. CRAO - cherry-red spot. PION - normal disc acutely. Optic neuritis - younger, painful.

Teaching Pearl

GCA is the ONLY vasculitis causing blindness - a true ophthalmic emergency. Risk to fellow eye = 25-50% within days WITHOUT treatment. Treat with steroids IMMEDIATELY - biopsy can wait 1-2 weeks (histology remains positive). Tocilizumab (IL-6 inhibitor, GiACTA trial) is the first approved biologic for GCA. Vision lost to arteritic AION rarely recovers (<15%).

Original answer transcript

History Workup
START STEROIDS IMMEDIATELY - do not wait for biopsy results. ESR (Westergren, often
Jaw claudication (most specific - ~65% sensitive), scalp tenderness, temporal artery >100) + CRP (more sensitive - elevated in nearly all active GCA). CBC (thrombocytosis,
tenderness/nodularity, PMR symptoms (proximal girdle stiffness worse in AM), prior anemia). Temporal artery biopsy (>=2 cm, within 1-2 weeks after starting steroids -
transient monocular VL, constitutional symptoms (fever, weight loss), diplopia, prior histology remains positive). Color duplex US (halo sign around temporal arteries). PET-CT
elevated ESR/CRP. for large-vessel involvement.
Findings Management
Acute: IV methylprednisolone 250-500 mg q6h if vision already lost (may protect fellow
Pale, swollen optic disc with CHALKY-WHITE PALLOR (more profound than NAION) and
eye). Oral prednisone 1 mg/kg/day if vision intact. Long-term: slow taper over 1-2 years
blurred margins. Possible segmental/altitudinal disc edema. Flame hemorrhages at disc guided by ESR/CRP. Tocilizumab (IL-6 inhibitor - GiACTA trial) = steroid-sparer, reduces
margin. Chalky-white disc = hallmark of arteritic AION. Concurrent cilioretinal artery relapse + cumulative steroid dose. Low-dose aspirin 81 mg. Annual CXR for aortic
occlusion possible.
aneurysm.
DDx
Arteritic AION (GCA) - top here: chalky disc + systemic symptoms + elevated ESR/CRP.
Non-arteritic AION (NAION) - small disc-at-risk, vasculopathic risk factors (HTN/DM/sleep
apnea), normal inflammatory markers, altitudinal field defect. CRAO - cherry-red spot.
PION - normal disc acutely. Optic neuritis - younger, painful.
TEACHING PEARL
GCA is the ONLY vasculitis causing blindness - a true ophthalmic emergency. Risk to fellow eye = 25-50% within days WITHOUT treatment. Treat with steroids
IMMEDIATELY - biopsy can wait 1-2 weeks (histology remains positive). Tocilizumab (IL-6 inhibitor, GiACTA trial) is the first approved biologic for GCA. Vision lost to
arteritic AION rarely recovers (<15%).