1. A Management Algorithm for Temporal Arteritis How Not to Miss this Blinding Disease Duncan P. Anderson, MD University of British Columbia Division of Neuro-Ophthalmology

2. 55 year old female 96 09 01: Frontal headache – acetaminophen 96 09 15: Diplopia, left ptosis, 20 minutes of blurred vision after bending/lifting 96 10 01: Increased headache (10/10), photophobia, diplopia, blurred vision, Left III palsy, dilated pupil, 20/100 OS Case Presentation, TA

3. 96 10 02: Admitted to hospital. Normal head CT head, normal fundi, blind OS Angiogram requested. ESR 28 Left III palsy, 20/20 – NLP, Left afferent + efferent pupil defects Ophthalmodynanometry 50/20 – 0/0 Left Central Retinal Artery Occlusion Admits decreased appetite, weight, jaw pain treated with i.v. methylprednisolone, heparin temporal artery biopsy requested Case Presentation, TA

5. 96 10 03: temporal artery biopsy positive 20/20 OD, no light perception OS ophthalmodynamometry 40/20 OD, 1/10 OS intraocular pressure 10mmHg OD, 2mmHg OS left ophthalmic artery occlusion, bilateral carotid stenosis 96 10 09: 20/20 OD, no light perception OS ophthalmodynamometry 40/20 OD, 10/5 OS intraocular pressure 15mmHg OD, 6mmHg OS treated with prednisone and coumadin Case Presentation, TA

7. 96 11 05: 20/20 OD, no light perception OS ophthalmodynanometry 70/30 OD, 35/10 OS intraocular pressure 16 OD, 12 OS mmHg left III palsy improving Prednisone 80 mg/day 97 11 05: stopped steroids Blurriness ]right eye, headache, ESR 42 Prednisone re-started at 60 mg/day 98 04 : tapered to Prednisone 10 mg/day Case Presentation, TA

9. HISTORY 91 year-old male awoke with decrease vision OD 6 days ago, involving superior field Bad vision OS due to infection at age of six Past history: hypertension, diabetes, well controlled No eye pain, headache, jaw claudication, muscle pain, fatigue, malaise, fever, temporal artery tenderness, pain on combing hair, or anorexia

10. EXAMINATION Visual acuity: 20/200 OD, 20/100 OS Right relative afferent pupil defect Fundus: pale swollen disc OD normal OS normal retinal artery pressure No temporal artery tenderness ESR 22mm/hr

12. Diagnosis –1.Nonarteritic anterior ischemic optic neuropathy RE - 2. left corneal scar No evidence to suggest temporal arteritis Treatment: prednisone 60 mg/day to reduce swelling for 5 days

13. 1 week after finished prednisone he developed decrease vision OS on awakening, now can’t get around the house No other symptoms of temporal arteritis VA: hand motion OD, light perception OS Fundus: pale flat right optic disc swollen pale left disc Diagnosis 1.Bilateral anterior ischemic optic neuropathy suspect arteritic cause

14. Plan: immediate temporal artery biopsy Rx: predisone 1000 mg/day x 2 day then taper off Temporal artery biopsy positive for arteritis ESR 34/hr Final visual acuity: count fingers OD, hand motion OS.

15. JW 85 YEAR OLD ♀ Sept 25Flashes & Blur OD 26Flashes & Blur OS ESR 71 – No arteritic symptoms i.v. methylprednisolone 1gm/day for 6 days then oral prednisone 100mg/day Oct 2ESR 24 TAB Positive 12Visual Hallucinations ESR 8 V HM

16. EP 77 YEAR OLD ♀ Late Augheadache, Fatigue, jaw claudication, weight loss Sept 23Blur OD 25ESR > 100 IV methylprednisolone 1gm/day x 3days 27Blur OS IV methylprednisolone 1gm/day x 3days oral prednisone 100mg/day Oct 2temporal artery biopsy positive 18tapered to prednisone 20mg/day V LP

24. AGE Prevalence of giant cell arteritis (%) 50 – 60 60 – 70 70 – 80 80 – 90 0.01 0.1 0.5 1.0

25. CLINICAL positive LR* negative LR Headache Jaw Claudication Abn. temporal artery Decreased Vision Diplopia Polymyalgia rheum. Fatigue/weight loss 1.5 5.4 3.1 1.3 3.2 1.0 1.3 1.0 0.9 1.0 0.9 1.0 * LR = Likelihood Ratio

26. LAB positive LR* negative LR ESR <50 50 – 100 >100 ↑ Platelets 0.6 1.1 2.5 6.0 1.6 0.9 0.8 0.6 *LR = Likelihood Ratio

27. TEMPORAL ARTERITIS GCA does not equal PMR symptoms to diagnosis: diagnosis to Biopsy: Arteritic ION without GCA symptoms: False Negative biopsy5% 3 – 4 mos 1 wk 20%

28. THINK Temporal Arteritis 1) Age > 50 2) Ischemic Optic Neuropathy 3) Amaurosis Fugax 4)ION with ↓↓ acuity/White Disc 5)ION with CRAO/Choroidal Ischemia 6) ↑ ESR, Creactive Protein, Platelets

29. TEMPORAL ARTERITIS 5 – 10% Arteritic ION lose acuity after Steroids (5d) 0.5% temporal arteritis lose acuity Post Steroids IV = PO Steroid Effect temporal arteritis can remain active ½ - 10 years Taper Steroids while following symptoms & ESR/CRP Re – Biopsy for Confirmation if necessary

32. TREATMENT p.o. Prednisone 80 mg/d 1 - 2 weeks 40 mg/d 2 - 3 months 10 mg/d 1 - 2 years

33. TREATMENT bilateral disease second eye progressive disease IV Methylprednisolone 1 gm/day for:

35. SUMMARY - TEMPORAL ARTERITIS Diagnosis: history temporal artery biopsy within 1 - 2 weeks Treatment: steroids (STAT) medical emergency taper slowly (mos) manage steroid complications switch to methotrexate

36. BIBLIOGRAPHY Niederkohr, R.D. & Levin, L.A. (2005). Management of the Patient with Suspected Temporal Arteritis: A Decision – Analytic Approach. Ophthalmology, 112(5), 744 – 1060. Younge, B.R., Cook Jr., B.E., Bartley, G.B., Hodge, D.O., Hunder, G.G. (2004). Initiation of Glucocorticoid Therapy: Before or After Temporal Artery Biopsy? Mayo Clin Proc, 79, 483 – 491. Hayreh, S.S., Zimmerman, B. (2003). Visual Deterioration in Giant Cell Arteritis Patients While on High Doses of Corticosteroid Therapy. Ophthalmology, 110(6), 1204 – 1215. Smetana, G.W., Shmerling, R.H. (2002). Does This Patient Have Temporal Arteritis? JAMA, 287(1), 92 – 101. Riordan-Eva, P., Landau, K., O’Day, J. (2001). Temporal artery biopsy in the management of giant cell arteritis with neuro-ophthalmic complications. Br J Ophthalmol, 85, 1248 – 1251. Hayreh, S.S., Podhajsky, P.A., Zimmerman, B. (1998). Ocular Manifestations of Giant Cell Arteritis. Am J Ophthalmol, 125(4), 509 – 520. Hayreh, S.S., Podhajsky, P.A., Zimmerman, B. (1998). Occult Giant Cell Arteritis: Ocular Manifestations. Am J Ophthalmol, 125(4), 521 – 526.