Eduovisual
Special Senses & Otolaryngology
Anterior ischemic optic neuropathy: arteritic vs non-arteritic
— Arteritic AION (A-AION): caused by giant cell arteritis (GCA), a granulomatous vasculitis of medium/large vessels in patients >50 (almost always >60)
— Non-arteritic AION (NAION): small-vessel/microvascular ischemia, usually in patients 50–70 with vasculopathic risk factors and a "disc at risk" (small, crowded cup)
— Sudden, painless, monocular vision loss noticed on awakening or within hours
— Altitudinal field defect (classically inferior) or central scotoma
— Relative afferent pupillary defect (RAPD) with optic disc edema
— Age ≥50, especially ≥70
— Jaw claudication, scalp tenderness, new temporal headache, polymyalgia rheumatica symptoms, weight loss, fever, anorexia
— Amaurosis fugax preceding permanent loss, diplopia, or contralateral eye involvement within days
— Profound vision loss (often counting fingers or worse), chalky-white pallid disc edema
— NAION incidence ~2–10/100,000 in patients >50; most common acute optic neuropathy in adults >50
— GCA incidence rises steeply after 70; untreated, second eye involved in 25–50% within days to weeks
Step 3 management: In any patient ≥50 with acute monocular vision loss, your first reflex is to stat ESR, CRP, and CBC with platelets, and if any clinical suspicion of GCA exists, start high-dose corticosteroids immediately — do not wait for temporal artery biopsy. Treatment delay is measured in hours, not days, because the fellow eye is at imminent risk.
Board pearl: "Pale, swollen disc + jaw claudication + ESR 100" = A-AION until proven otherwise; "hyperemic disc edema + small cup + HTN/DM, vision 20/60" = NAION.
— Both A-AION and NAION present as acute, painless, monocular vision loss, typically over minutes to hours
— Often noticed upon awakening (nocturnal hypotension is a proposed NAION trigger)
— Stepwise/progressive worsening over 1–2 weeks in ~30% of NAION cases
— Altitudinal field defect (inferior > superior) is classic for both
— Central, arcuate, or cecocentral scotomas also possible
— Acuity:
— NAION: variable, often 20/60–20/200, sometimes near-normal
— A-AION: typically severe (≤20/200, often count fingers, hand motion, or no light perception)
— New headache (often temporal, but can be occipital/diffuse)
— Jaw claudication — pain in masseter with chewing (highest LR+ for GCA, ~4–9)
— Scalp tenderness (combing hair, lying on pillow)
— Transient monocular vision loss (amaurosis fugax) in prior days/weeks
— Diplopia from cranial nerve ischemia
— Polymyalgia rheumatica: proximal shoulder/hip girdle stiffness, worse in morning (~50% overlap with GCA)
— Constitutional: low-grade fever, fatigue, weight loss, night sweats, anorexia
— Tongue or limb claudication (rare but specific)
— HTN, diabetes, hyperlipidemia, smoking, obstructive sleep apnea
— Nocturnal hypotension from aggressive evening antihypertensives
— PDE-5 inhibitor use (sildenafil/tadalafil) — controversial but association reported
— Amiodarone (rare association)
— Recent cataract surgery, severe acute hypotension/blood loss, hemodialysis
Key distinction: A-AION usually has systemic symptoms preceding or accompanying vision loss; NAION patients feel systemically well with only an eye complaint.
Board pearl: A patient with a recent transient ipsilateral vision blackout lasting minutes ("the curtain came down then lifted") followed days later by permanent vision loss = GCA prodrome — biopsy and steroids.
— Check each eye separately; document Snellen and pinhole
— Dyschromatopsia (red desaturation) out of proportion to acuity loss → optic nerve disease
— Relative afferent pupillary defect (RAPD) in affected eye — swinging flashlight test
— Bilateral AION may mask RAPD (look for sluggish reactions)
— Altitudinal defect respecting the horizontal meridian is highly suggestive
— Formal Humphrey or Goldmann perimetry confirms and quantifies
— NAION: small, crowded optic disc with absent or tiny cup ("disc at risk"); hyperemic, segmental or diffuse swelling; splinter hemorrhages at disc margin
— A-AION: chalky-white, pallid edema of the disc; cotton-wool spots; sometimes cilioretinal artery occlusion
— Fellow eye in NAION classically shows a small cup-to-disc ratio (<0.2); fellow eye in A-AION has a normal cup
— Palpate the temporal arteries: nodular, beaded, tender, thickened, or pulseless artery
— Scalp tenderness on palpation
— Auscultate carotids and subclavians (large-vessel GCA can cause bruits, BP asymmetry)
— Check BP in both arms — >10 mmHg difference suggests large-vessel involvement
— Orthostatic vitals (postural hypotension may suggest overaggressive antihypertensive dosing)
— Volume status, recent surgery/blood loss
— Screen for OSA features (Mallampati, BMI, neck circumference, STOP-BANG)
CCS pearl: Order "funduscopic exam" and "temporal artery palpation" explicitly in your CCS workup of acute monocular vision loss; both contribute to localization and probability of GCA.
Board pearl: Pallid disc edema in a 75-year-old = arteritic until proven otherwise.
— ESR: classically elevated; use age-adjusted upper limit
— Men: age/2; Women: (age+10)/2
— ESR >50 is suspicious; ESR often >80–100 in active GCA
— CRP: more sensitive than ESR; elevated in >95% of GCA
— Combination of elevated ESR + CRP has the best diagnostic performance (sensitivity ~97%)
— Note: ~4% of biopsy-proven GCA has normal ESR and CRP
— Thrombocytosis (>400) is a strong supportive finding in GCA (chronic inflammation)
— Normocytic anemia of chronic disease common
— Baseline glucose, creatinine, LFTs (alkaline phosphatase can be elevated in GCA)
— Needed before initiating high-dose steroids
— Fasting lipid panel, HbA1c, BP measurement
— Sleep study referral if OSA features
— Medication review (timing of antihypertensives, PDE-5 inhibitors, amiodarone)
— MRI brain and orbits with contrast and fat suppression if diagnosis uncertain — rules out compressive, infiltrative, demyelinating optic neuropathy
— MRI of optic nerve in optic neuritis shows enhancement of nerve; AION typically shows nerve head swelling without retrobulbar enhancement
— Consider vascular imaging (MRA, CTA, or temporal artery ultrasound) in suspected large-vessel GCA
— Temporal artery ultrasound: "halo sign" (hypoechoic wall thickening) — increasingly used; sensitivity ~70%, specificity ~95% in expert hands
Step 3 management: Order ESR, CRP, CBC stat in any patient ≥50 with acute optic neuropathy — results within hours guide whether to commit to long-term steroids.
Board pearl: Normal ESR does not exclude GCA; rely on the combination of ESR + CRP + platelets + clinical features.
— Obtain a ≥1–2 cm segment (ideally 2 cm) of the symptomatic-side superficial temporal artery
— Skip lesions are common → adequate length improves yield; contralateral biopsy if first is negative and suspicion remains high
— Do NOT delay steroid initiation while awaiting biopsy — biopsy remains positive for at least 2 weeks (often 4+ weeks) after starting steroids
— Histology: granulomatous inflammation, multinucleated giant cells, fragmented internal elastic lamina, intimal hyperplasia
— Sensitivity ~75–85%; a negative biopsy with high clinical suspicion still warrants treatment (clinically diagnosed GCA)
— "Halo sign" (non-compressible hypoechoic mural thickening) and "compression sign"
— EULAR now recommends ultrasound as first-line imaging in suspected cranial GCA in experienced centers; may obviate biopsy if classic
— CTA, MRA, or FDG-PET/CT of aorta and great vessels
— Findings: aortic wall thickening, aortitis, subclavian/axillary stenosis
— Up to 25% of GCA has large-vessel involvement; predisposes to thoracic aortic aneurysm
— Delayed choroidal filling is characteristic of A-AION (posterior ciliary occlusion)
— NAION typically shows preserved choroidal filling
— Humphrey 24-2 documents altitudinal/arcuate defects; baseline for monitoring
— MRI brain/orbits with contrast if young patient, painful, or atypical → rule out optic neuritis, compressive lesion, infiltrative disease
— Serologies (RPR, ACE, ANA, ANCA, Lyme) for atypical optic neuropathies
— Carotid Doppler if embolic phenomenon (overlapping CRAO)
CCS pearl: In CCS, after starting IV methylprednisolone, the next orders are "temporal artery biopsy, schedule" and "ophthalmology consult" — biopsy within 1–2 weeks preserves diagnostic yield.
Key distinction: Biopsy confirms GCA; clinical decision to treat does not wait for it.
— Any patient ≥50 with acute monocular vision loss → assume GCA possible until disproven
— Pallid disc edema, severe vision loss, or systemic GCA symptoms → treat empirically while working up
— Required: age ≥50 with diagnosis of medium/large vessel vasculitis
— Clinical: morning stiffness in shoulders/neck, sudden visual loss, jaw/tongue claudication, new temporal headache, scalp tenderness, abnormal temporal artery exam
— Lab/imaging: ESR ≥50 or CRP ≥10, positive TAB or halo sign on ultrasound, bilateral axillary involvement, FDG-PET activity in aorta
— High probability (classic symptoms + elevated markers + age >70 + pallid disc edema) → IV methylprednisolone immediately, biopsy within days
— Intermediate probability → oral prednisone 1 mg/kg/day while expediting biopsy/ultrasound
— Low probability (typical NAION features, no GCA red flags, normal markers) → manage as NAION; address risk factors
— No proven acute treatment reverses vision loss
— Avoid harm from misdiagnosed GCA → still check ESR/CRP
— Optimize vasculopathic risk factors to reduce fellow-eye risk (~15–25% over 5 years)
— Review medications: shift antihypertensives to morning, discuss PDE-5 inhibitor risk-benefit
— Evaluate and treat OSA (CPAP may reduce fellow-eye involvement)
— A-AION → admit for IV pulse steroids in many centers; outpatient if reliable, monitored
— NAION → outpatient ophthalmology follow-up within 1–2 weeks
Step 3 management: The single most important decision in acute AION evaluation is "Is this GCA?" — getting this right prevents bilateral blindness and stroke.
Board pearl: When in doubt, treat for GCA. The risk of unnecessary steroids for 1–2 weeks is far less than the risk of bilateral blindness from missed GCA.
— With vision loss or threatened vision (amaurosis, diplopia, fellow-eye involvement):
— IV methylprednisolone 1 g daily × 3 days, then transition to oral prednisone
— Without ocular involvement (cranial GCA without ischemia, PMR with GCA features):
— Oral prednisone 1 mg/kg/day (max 60–80 mg) for 2–4 weeks, then taper
— Taper guided by symptoms and inflammatory markers (ESR, CRP):
— Reduce by ~10 mg every 2 weeks down to 20 mg
— Then by 2.5 mg every 2–4 weeks down to 10 mg
— Then by 1 mg every 1–2 months
— Total duration typically 1–2 years, often longer
— Relapse rate is high (~50%) during taper; treat with steroid bump
— Tocilizumab (IL-6 receptor antagonist): SC 162 mg weekly; FDA-approved for GCA, reduces cumulative steroid exposure and relapse rate (GiACTA trial)
— Methotrexate has modest evidence as adjunct
— Low-dose aspirin 81 mg daily — observational data suggest reduced ischemic events (vision loss, stroke); some controversy, but commonly given
— PPI for GI prophylaxis on high-dose steroids
— Calcium 1200 mg + vitamin D 800 IU daily + bisphosphonate for steroid-induced osteoporosis prophylaxis (any patient expected to be on ≥7.5 mg prednisone for ≥3 months)
— PJP prophylaxis (TMP-SMX) if prednisone ≥20 mg/day for >4 weeks plus second immunosuppressant
— Vaccinations updated before immunosuppression when possible (avoid live vaccines once on steroids)
— Aspirin 81 mg daily is often given to reduce fellow-eye vascular events (modest evidence)
— Optimize HTN, DM, lipids, OSA
— Avoid evening dosing of antihypertensives
— Counsel on PDE-5 inhibitor association
— Systemic steroids in NAION are not recommended (no proven benefit, harms exist)
Board pearl: Tocilizumab + tapering steroids is now standard for relapsing or steroid-toxic GCA — know this for Step 3.
Step 3 management: Every GCA patient on chronic steroids needs bone protection, GI prophylaxis, glucose monitoring, BP monitoring, and vaccination review.
— Performed by vascular surgery, plastic surgery, ophthalmology, or otolaryngology
— Outpatient, local anesthesia, ~1-hour procedure
— Sample ≥1–2 cm of symptomatic temporal artery; skip lesions favor longer specimens
— Biopsy within 1–2 weeks of starting steroids preserves diagnostic yield; up to 4 weeks still acceptable
— Complications (rare): wound infection, scalp necrosis (if extensive collateral compromise), CN VII frontal branch injury
— Bilateral biopsy considered if first is negative and clinical suspicion remains high (yield gain ~5%)
— In experienced centers, temporal/axillary artery ultrasound may replace biopsy when classic halo sign present with high pretest probability
— Non-invasive, immediate, repeatable
— Optic nerve sheath decompression is NOT beneficial — the Ischemic Optic Neuropathy Decompression Trial (IONDT) showed harm: worse visual outcomes and higher complication rates
— Board favorite negative trial — know it
— Post-operative NAION is a recognized complication, particularly in eyes with small cups
— Defer second-eye cataract surgery if NAION occurred after the first
— Aortic aneurysm surveillance with annual chest imaging (CXR or CT) — thoracic aortic aneurysm risk is 17-fold increased
— Aneurysm repair per standard vascular criteria
— Subclavian/axillary stenosis with limb claudication may rarely require endovascular intervention
— Low-vision rehabilitation referral
— Driving evaluation when monocular vision affects fitness
— Occupational therapy for ADL adaptation
Board pearl: The IONDT trial definitively buried optic nerve sheath decompression for NAION — if a stem offers it, the answer is no.
CCS pearl: Order "temporal artery biopsy" and "ophthalmology consult" in CCS for suspected GCA; do not order it in NAION.
— GCA is essentially a disease of patients ≥50, with mean age at onset ~72
— Atypical GCA presentations are common in the very elderly:
— Fever of unknown origin (GCA causes 15% of FUO in patients >65)
— Isolated weight loss or anorexia
— Cognitive decline or stroke as presenting feature
— Lower threshold to image extracranial vessels in elderly with constitutional symptoms
— Hyperglycemia and new-onset diabetes — check glucose at baseline, weekly initially
— Hypertension exacerbation — uptitrate antihypertensives
— Osteoporosis and fragility fracture — DEXA at baseline; start bisphosphonate (or denosumab if eGFR <35) early
— Steroid-induced myopathy and falls — physical therapy
— Mood and cognitive effects — insomnia, psychosis, delirium, especially with IV pulse
— Skin atrophy, bruising, poor wound healing
— Infection risk — vaccinate (inactivated influenza, pneumococcal PCV20, RSV, shingles non-live recombinant, COVID-19); avoid live vaccines once immunosuppressed
— Steroid dosing unchanged
— Tocilizumab: caution with severe renal impairment (limited data, generally usable)
— Bisphosphonates: avoid IV zoledronate if eGFR <35; use denosumab as alternative
— Watch NSAIDs and contrast carefully in pre-renal/CKD elderly
— GCA itself can elevate alkaline phosphatase and transaminases (resolves with treatment)
— Tocilizumab can cause transaminitis — monitor LFTs at baseline, every 4–8 weeks
— Avoid tocilizumab if active hepatic disease
— Review for QT-prolonging drugs (especially if vision loss raised concern for embolic stroke workup)
— Evening antihypertensives → shift to morning to mitigate nocturnal hypotension–induced NAION
Step 3 management: Every elderly GCA patient starting prednisone gets a bundle: glucose check, BP check, DEXA, calcium/vitamin D, PPI, bisphosphonate, vaccination review, fall-risk assessment.
Board pearl: New-onset FUO + headache + anemia + elevated ESR in a 75-year-old = GCA workup.
— Both A-AION (GCA) and classic NAION are rare in children
— Pediatric AION usually associated with:
— Severe acute blood loss or hypotension (perioperative, trauma)
— Cardiac surgery
— Hemodialysis-associated hypotension
— Migraine, hypercoagulable states
— GCA does not occur in children; pediatric large-vessel vasculitis is Takayasu arteritis — consider in adolescents/young adults with limb claudication, BP asymmetry, bruits
— GCA in pregnancy is exceedingly rare (age mismatch)
— Pregnant patients with optic neuropathy → think optic neuritis (MS, NMO), preeclampsia/eclampsia-related cortical vision changes, pituitary apoplexy (Sheehan), or cerebral venous sinus thrombosis
— Steroids if needed in pregnancy: prednisone preferred (minimally crosses placenta); dexamethasone/betamethasone cross more freely
— Strongly consider alternatives: optic neuritis (MS, NMO-IgG, MOG), Leber hereditary optic neuropathy (LHON), toxic/nutritional optic neuropathy, infiltrative or compressive lesion
— Order MRI brain/orbits with contrast, AQP4-IgG, MOG-IgG, mitochondrial DNA testing as appropriate
— Counsel that NAION has been associated with PDE-5 inhibitor use, especially in patients with a "disc at risk"
— If NAION occurs in one eye, discontinue PDE-5 inhibitor to reduce fellow-eye risk
— GCA is markedly more common in Northern European descent; rare in Black, Asian, and Hispanic populations
— But never use ethnicity alone to exclude — confirm with labs and biopsy if clinical suspicion
— Perioperative ION (often posterior, not anterior) after prolonged prone spine surgery is a recognized entity → optimize positioning, hemodynamics, hematocrit
— Cardiac surgery patients can develop NAION from intraoperative hypotension
Key distinction: "Optic neuropathy in a 30-year-old woman with eye pain on movement" → optic neuritis, not AION. "Painless optic neuropathy in a 70-year-old man with jaw claudication" → GCA.
Board pearl: Pediatric large-vessel vasculitis = Takayasu; geriatric large-vessel vasculitis = GCA.
— Permanent visual loss is the rule, not the exception
— A-AION: severe loss in affected eye; without treatment, 25–50% develop fellow-eye involvement within days to weeks
— NAION: stable or slow recovery in 30–40%; fellow-eye risk ~15–25% over 5 years
— Optic atrophy develops over 4–8 weeks (pale disc on follow-up exam)
— No light perception outcomes are more common in A-AION than NAION
— Stroke — vertebrobasilar more than carotid; risk ~3–7%
— Aortic aneurysm and dissection — thoracic > abdominal; 17× increased risk for thoracic; surveillance imaging annually
— Limb claudication from subclavian/axillary involvement
— Myocardial infarction from coronary vasculitis (rare)
— Scalp and tongue necrosis in severe untreated disease
— Cranial neuropathies (especially diplopia from CN III, IV, VI involvement)
— Hyperglycemia, new-onset diabetes
— Osteoporosis, vertebral compression fractures
— Cataract and steroid-induced glaucoma
— Infection (bacterial pneumonia, PJP, reactivation TB, herpes zoster)
— Avascular necrosis of hip/shoulder
— Mood disturbance, psychosis, insomnia
— Weight gain, Cushingoid features
— Skin fragility, easy bruising, poor wound healing
— Adrenal suppression with abrupt withdrawal
— Neutropenia, transaminitis, hyperlipidemia
— GI perforation (especially with concurrent NSAID/steroid, diverticular disease)
— Increased infection risk
— Masks CRP — relapse must be detected clinically, not by CRP
— Loss of driving, independence, employment
— Depression and anxiety after sudden vision loss
Board pearl: Once on tocilizumab, CRP and ESR are unreliable disease activity markers — monitor symptoms.
Step 3 management: All GCA patients need lifetime aortic surveillance (annual chest imaging) regardless of remission status.
— Any acute monocular vision loss
— Document baseline acuity, fields, fundus
— Coordinate biopsy, perimetry, OCT
— Acute vision loss or amaurosis fugax → admit for IV pulse methylprednisolone, expedited biopsy, monitoring
— Bilateral vision involvement or fellow-eye threat
— Stroke symptoms (vertebrobasilar TIA, posterior circulation deficits)
— Diplopia or new cranial neuropathy
— Severe systemic illness, unable to tolerate oral intake
— Diagnostic uncertainty requiring rapid multispecialty workup
— Outpatient pulse-steroid logistics unavailable
— Rheumatology — long-term GCA management, tocilizumab initiation, steroid sparing
— Vascular surgery / plastics / ENT — temporal artery biopsy
— Neurology / stroke service — if stroke symptoms or cranial neuropathy
— Cardiothoracic surgery — if aortic aneurysm identified
— Endocrinology — if pre-existing diabetes or brittle glycemic control on steroids
— Sleep medicine — for NAION patients with OSA features
— Concurrent posterior circulation stroke with airway/swallowing compromise
— Aortic dissection
— Steroid psychosis with safety risk
— Almost always outpatient management
— Urgent (not emergent) ophthalmology referral within 1–2 weeks
— PCP follow-up within 1–2 weeks for risk factor optimization
— Sleep study referral
— Counsel patient on stroke-equivalent risk reduction
— Clear written discharge instructions: steroid dosing, taper schedule, prophylactic meds, warning signs (fellow-eye blurring → return immediately)
— Confirm follow-up appointments before discharge
— Provide rheumatology appointment within 1–2 weeks
CCS pearl: In CCS for suspected GCA, sequence: IV methylprednisolone → ESR/CRP/CBC → ophthalmology consult → admit → temporal artery biopsy → rheumatology consult → bone protection bundle → discharge planning with taper schedule.
Board pearl: Acute vision loss + suspected GCA = admit + IV pulse steroids, even before biopsy.
— Infarction of retrobulbar optic nerve → acute vision loss with normal fundus initially, optic atrophy weeks later
— Causes: GCA, perioperative (prone spine surgery), severe hypotension/anemia
— No disc edema = key differentiator from AION
— Younger patients (15–45), often female
— Pain with eye movement (90%), central scotoma, dyschromatopsia
— Disc usually normal acutely (retrobulbar) in MS-related; swollen in NMO/MOG
— MRI brain/orbits → enhancing optic nerve; brain lesions in MS
— Treat with IV methylprednisolone 1 g × 3–5 days then taper
— Acute, painless, profound monocular vision loss
— Fundus: pale retina with cherry-red spot, boxcar segmentation
— RAPD present; disc not swollen initially
— Embolic source workup (carotid Doppler, echo, ECG/Holter, hypercoagulability)
— GCA causes ~5% of CRAO — check ESR/CRP
— Painless vision loss; fundus shows "blood and thunder" — diffuse hemorrhages in all 4 quadrants, dilated tortuous veins
— HTN, glaucoma, hyperviscosity
— Floaters, sudden visual blur/loss
— Often diabetic proliferative retinopathy or retinal tear
— Flashes, floaters, curtain across vision, peripheral field defect
— Surgical emergency
— Bilateral or unilateral disc edema in diabetic patients, mild vision loss
— Distinguishable by demographics and milder course
— Young males, painless sequential bilateral vision loss
— Mitochondrial DNA mutations
— Ethambutol, methanol, B12 deficiency → painless bilateral central scotomas
Key distinction: Painful + young + eye movement = optic neuritis; painless + elderly + pallid disc + ↑ESR = A-AION; painless + elderly + cherry-red spot = CRAO.
Board pearl: Always check ESR/CRP in any acute optic or retinal ischemic event in patients >50 — GCA hides in CRAO and PION too.
— Occipital stroke → homonymous hemianopia with macular sparing, both eyes affected
— Patient may misperceive as monocular if not carefully tested
— No RAPD, normal fundus, normal acuity if macula spared
— Workup: stat CT/MRI brain, stroke pathway
— Brief monocular vision loss ("curtain coming down") lasting minutes
— Carotid stenosis, cardioembolic, or GCA prodrome
— Workup: carotid Doppler, echo, ECG/telemetry, ESR/CRP, lipid panel, HbA1c
— Sudden headache, vision loss (often bitemporal hemianopia), ophthalmoplegia, hypopituitarism
— MRI sella; emergent neurosurgical and endocrine consultation
— Hydrocortisone empirically for adrenal crisis
— Visual scintillations, fortification spectra, lasting <60 minutes, followed by headache
— History of similar episodes
— Young, obese woman with headaches, transient visual obscurations, bilateral papilledema, pulsatile tinnitus
— Lumbar puncture: elevated opening pressure
— Inconsistent exam, normal pupils, normal fundus, normal OCT
— Diagnosis of exclusion
— Neck pain, Horner syndrome, transient vision changes, stroke risk
— CTA neck
— Cortical vision changes, hypertension, seizures, headache
— MRI shows posterior parieto-occipital edema
— Acute bilateral vision loss, metabolic acidosis with high anion gap
— Treat with fomepizole, dialysis
Step 3 management: Acute "vision loss" workup should always include neurologic exam and consideration of stroke, not just an ocular workup — call stroke alert if focal deficits exist.
Board pearl: Bitemporal hemianopia → think chiasmal lesion (pituitary apoplexy); homonymous hemianopia → occipital stroke; altitudinal defect → AION.
— Prednisone taper over 12–24 months, guided by symptoms + ESR/CRP (until on tocilizumab)
— Tocilizumab 162 mg SC weekly for steroid-sparing in relapsers or high steroid-toxicity risk patients
— Aspirin 81 mg daily for reduction of ischemic events (per many rheumatology guidelines)
— Bone protection:
— Calcium 1200 mg/day + vitamin D 800–1000 IU/day
— Bisphosphonate (alendronate, risedronate) for any patient on prednisone ≥7.5 mg/day for ≥3 months
— Denosumab if CKD; teriparatide for severe osteoporosis
— DEXA baseline and every 1–2 years
— GI protection: PPI while on high-dose steroids
— Glycemic monitoring: home glucose log; HbA1c every 3 months
— BP monitoring: home BP log; uptitrate antihypertensives
— Vaccinations: inactivated influenza annually, PCV20, recombinant zoster (Shingrix), RSV (≥60), COVID-19 boosters, Tdap; avoid live vaccines while immunosuppressed
— PJP prophylaxis (TMP-SMX) if prednisone ≥20 mg/day plus second immunosuppressant for prolonged period
— Annual chest imaging for aortic aneurysm surveillance (CT or CXR; many use chest CT every 2–3 years)
— Aggressive vascular risk modification — treat as cardiovascular equivalent
— BP <130/80
— LDL <70 if ASCVD, <100 otherwise; statin per AHA/ACC ASCVD risk
— HbA1c <7% in most diabetics
— Smoking cessation, weight loss, exercise
— Aspirin 81 mg daily — modest evidence to reduce fellow-eye risk
— CPAP for OSA if confirmed — may reduce fellow-eye involvement
— Shift antihypertensives to morning to avoid nocturnal hypotension
— Discuss/discontinue PDE-5 inhibitors in patients with NAION
— Low-vision rehabilitation referral
Step 3 management: Discharge bundle for GCA = prednisone + taper plan + ASA + Ca/VitD + bisphosphonate + PPI + vaccinations + rheumatology follow-up + biopsy report follow-up + aortic surveillance schedule.
Board pearl: NAION is a vascular disease of the optic nerve — manage the patient like you would a stroke survivor.
— Week 1–2: rheumatology evaluation, biopsy results review, baseline DEXA
— Weeks 2–4: symptom review, ESR/CRP, glucose, BP; begin taper if controlled
— Months 1–6: every 2–4 weeks — ESR/CRP, CBC, CMP, symptom assessment, taper adjustment
— Months 6–24: every 1–3 months
— Beyond 2 years (if remission): every 6–12 months with ongoing aortic surveillance
— Annual chest imaging for aortic aneurysm (CT chest q2–3 years commonly)
— Tocilizumab monitoring: CBC, LFTs, lipids every 4–8 weeks initially
— Ophthalmology at 1–2 weeks, 1 month, 3 months, then annually
— Document visual field, OCT, acuity changes
— PCP visit at 1–2 weeks to optimize vascular risk factors
— Sleep study within 1–3 months if OSA suspected
— Low-vision specialist referral for magnifiers, adaptive technology, lighting
— Occupational therapy for ADL and home safety
— Orientation and mobility training if severe loss
— Driving assessment — most states require reporting vision below threshold; help patient transition
— Vocational counseling for working-age patients
— Mental health screening — depression is common after sudden vision loss
— Any new blurring, blackout, or vision change in the fellow eye → emergency department immediately
— Daily Amsler grid or covered-eye check for fellow-eye monitoring
— In GCA: any return of headache, jaw claudication, scalp tenderness, or new vision symptom → contact rheumatology urgently
— Steroid card for hospitalizations/anesthesia (adrenal insufficiency risk)
— Never stop steroids abruptly
— Annual flu vaccine, pneumonia vaccines, Shingrix
CCS pearl: Schedule "counsel patient, medication compliance", "counsel patient, smoking cessation", and "counsel patient, exercise" as part of NAION discharge.
Board pearl: Fellow-eye involvement is the most feared, preventable complication — counsel every patient to seek immediate care for any new visual symptom.
— Standard of care in suspected GCA is to start treatment immediately, with informed consent for empiric therapy
— Document discussion of risks (steroid adverse effects) vs benefits (vision and stroke prevention)
— Acceptable to start before biopsy; biopsy yield remains adequate for 1–2 weeks
— Most common malpractice issue in GCA is missed or delayed diagnosis leading to bilateral blindness
— Always check ESR + CRP in any patient ≥50 with new headache, jaw pain, vision change, or unexplained constitutional symptoms
— Document negative review of systems specifically
— Many states have mandatory or permissive physician reporting of vision loss below driving standards (typically <20/40 best-corrected or significant field loss)
— Know your state's requirements; document counseling about driving cessation
— Refer to DMV-affiliated low-vision evaluator if uncertain
— Discuss diabetes risk, infection risk, osteoporosis, mood changes, weight gain, cataract, AVN
— Discuss expected duration (often years) and taper plan
— Discuss steroid-sparing options (tocilizumab) and shared decision-making
— Most dangerous handoff: ED → home in suspected GCA without rheumatology follow-up secured
— Ensure first prednisone dose given in ED, biopsy appointment scheduled, rheumatology appointment within 1–2 weeks, written taper plan, emergency return instructions
— Closed-loop communication with PCP and ophthalmology
— Steroid + bisphosphonate + PPI + insulin + antihypertensives → med reconciliation and pillbox/family support
— Fall risk increases with steroid myopathy and visual impairment
— Counsel patient and family that abrupt steroid cessation can be fatal
— Provide steroid card; instructions for stress dosing during illness or surgery
— Coordinate with surgeons preoperatively for stress-dose hydrocortisone
Step 3 management: Suspected GCA discharged from ED without first-dose steroids and rheumatology follow-up is a patient safety event — treat like a STEMI being sent home without aspirin.
Board pearl: Never delay steroids for biopsy in GCA — this is both clinical standard and medicolegal expectation.
— Polymyalgia rheumatica — 40–50% overlap; treat with low-dose prednisone (15–20 mg) unless GCA features
— HLA-DRB1*04 genetic association
— Northern European descent; women 2–3× more than men
— Mean age 72; almost never <50
— Jaw claudication (LR+ ~6)
— New temporal headache (LR+ ~3)
— Scalp tenderness
— Diplopia (LR+ ~3)
— Beaded, tender, pulseless temporal artery
— ESR >100, CRP markedly elevated, thrombocytosis
— Pallid disc edema
— "Disc at risk" — small cup-to-disc ratio <0.2
— Vasculopathic risk factors: HTN, DM, dyslipidemia, smoking, OSA
— Nocturnal hypotension, PDE-5 inhibitors, amiodarone
— Onset on awakening
— Hyperemic disc edema (vs pallid in arteritic)
— IONDT — optic nerve sheath decompression harmful in NAION
— GiACTA — tocilizumab effective steroid-sparing in GCA
— Granulomatous inflammation with giant cells, intimal hyperplasia, fragmented elastic lamina — temporal artery biopsy
— Skip lesions; sample length matters
— Altitudinal defect respecting horizontal meridian = AION (either type)
— Delayed choroidal filling → A-AION
— 17× thoracic aortic aneurysm risk; annual chest imaging
— ESR can be normal in 4% of GCA — CRP and clinical picture matter
— Steroids may shrink temporal artery biopsy yield only after 2–4 weeks
— Tocilizumab suppresses CRP — monitor symptoms instead
— "Pale and old" = pallid disc + elderly = A-AION
— "Pink and packed" = hyperemic disc + small cup = NAION
Board pearl: If the stem mentions chalky-white disc edema or vision worse than 20/200 in a 75-year-old, the answer involves immediate IV steroids and temporal artery biopsy.
Key distinction: A-AION = vasculitis, pallid, severe loss, treat with steroids; NAION = microvascular, hyperemic, moderate loss, manage risk factors.
— 74-year-old woman with sudden painless vision loss in right eye on awakening. 3-week history of new bitemporal headache, jaw pain with chewing, low-grade fevers, 4-kg weight loss. Exam: vision count fingers OD, RAPD, chalky-white swollen disc. ESR 102, CRP 8.5, platelets 520.
— Next best step: IV methylprednisolone 1 g daily × 3 days, then transition to oral prednisone, schedule temporal artery biopsy within 1 week, rheumatology consult
— 62-year-old man with HTN, DM2, hyperlipidemia notes blurred vision in left eye on awakening. Vision 20/80, inferior altitudinal defect, hyperemic swollen disc, fellow disc with small cup. ESR 18, CRP 0.4.
— Next best step: optimize vascular risk factors, aspirin 81 mg daily, sleep study for OSA, ophthalmology follow-up; do not give steroids
— Patient with suspected GCA, biopsy scheduled in 5 days. Should steroids be delayed?
— Answer: No — start immediately; biopsy yield preserved 1–2 weeks (often longer)
— Patient with NAION on evening lisinopril and tamsulosin. What modification?
— Answer: Shift antihypertensives to morning; reduce nocturnal BP drop
— Patient develops NAION 24 hours after taking sildenafil. Counseling?
— Answer: Discontinue PDE-5 inhibitor to reduce fellow-eye risk
— TAB negative, but classic GCA features and ESR 95.
— Answer: Continue steroids (clinically diagnosed GCA); consider contralateral biopsy; rheumatology management
— Steroid toxicity → initiate tocilizumab for steroid-sparing
— Patient 5 years post-GCA diagnosis. What annual screening?
— Answer: Annual chest imaging for thoracic aortic aneurysm
— NAION patient asks about surgery. Answer: not recommended (IONDT)
— Patient starting 60 mg prednisone for 6 months. Prophylaxis?
— Answer: Calcium + vitamin D + bisphosphonate plus DEXA
Step 3 management: Recognize the "60+ year-old with painless vision loss and a constellation of systemic symptoms" stem — pivot immediately to ESR/CRP and steroids.
Board pearl: The most common right answer in a GCA stem is "start IV methylprednisolone now."
Anterior ischemic optic neuropathy is acute infarction of the optic nerve head presenting as sudden painless monocular vision loss — distinguish arteritic AION (giant cell arteritis), which demands immediate high-dose corticosteroids and temporal artery biopsy to prevent bilateral blindness and stroke, from non-arteritic AION, which is managed by aggressive vascular risk factor modification because no acute treatment reverses the visual loss.
— A-AION: age >70, pallid disc edema, severe vision loss (often <20/200), jaw claudication, scalp tenderness, new headache, ESR >50, CRP elevated, platelets >400
— NAION: age 50–70, hyperemic disc edema, moderate vision loss (often 20/60–20/200), small "disc at risk," vasculopathic risk factors, normal inflammatory markers
— Any age ≥50 with acute optic neuropathy → stat ESR + CRP + CBC + ophthalmology consult
— Suspected GCA → IV methylprednisolone 1 g × 3 days then oral prednisone; temporal artery biopsy within 1–2 weeks (do not delay treatment); aspirin 81 mg
— Confirmed NAION → optimize HTN/DM/lipids, morning antihypertensives, evaluate for OSA, aspirin 81 mg, counsel against PDE-5 inhibitors
— GCA: long taper (1–2 years), tocilizumab for steroid-sparing, bone protection bundle, vaccinations, annual aortic surveillance for life
— NAION: cardiovascular-equivalent risk reduction, low-vision rehabilitation, fellow-eye monitoring (15–25% lifetime risk)
— Patient safety: never discharge suspected GCA without first-dose steroids and rheumatology follow-up
— Delaying steroids for biopsy in suspected GCA
— Optic nerve sheath decompression for NAION (IONDT — harmful)
— Anchoring on "isolated headache" in elderly without checking ESR/CRP
— Stopping steroids abruptly (adrenal crisis risk)
Board pearl: The single most exam-tested decision in acute optic neuropathy is "is this GCA?" — if yes, steroids now, biopsy soon, taper over a year, tocilizumab if relapses, aortic imaging forever.