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 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 • 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 Case Presentation, TA temporal artery biopsy requested • 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 Case Presentation, TA treated with prednisone and coumadin • 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 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 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 • 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 • 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 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. JW 85 YEAR OLD ♀ Sept 25 Flashes & Blur OD 26 Flashes & Blur OS ESR 71 – No arteritic symptoms i.v. methylprednisolone 1gm/day for 6 days then oral prednisone 100mg/day Oct 2 ESR 24 TAB Positive 12 HM V HM Visual Hallucinations ESR 8 EP 77 YEAR OLD ♀ Late Aug Sept 23 25 headache, Fatigue, jaw claudication, weight loss Blur OD ESR > 100 IV methylprednisolone 1gm/day x 3days 27 Blur OS IV methylprednisolone 1gm/day x 3days oral prednisone 100mg/day Oct 2 18 temporal artery biopsy positive LP V tapered to prednisone 20mg/day LP AGE Prevalence of giant cell arteritis (%) 50 – 60 0.01 60 – 70 0.1 70 – 80 0.5 80 – 90 1.0 CLINICAL Headache positive LR* negative LR 1.5 1.0 Jaw Claudication 5.4 0.9 Abn. temporal artery 3.1 0.9 Decreased Vision 1.3 1.0 Diplopia 3.2 1.0 Polymyalgia rheum. 1.0 0.9 Fatigue/weight loss 1.3 1.0 * LR = Likelihood Ratio LAB positive LR* negative LR ESR <50 0.6 1.6 50 – 100 1.1 0.9 >100 2.5 0.8 ↑ Platelets 6.0 0.6 *LR = Likelihood Ratio TEMPORAL ARTERITIS • GCA does not equal PMR • symptoms to diagnosis: 3 – 4 mos • diagnosis to Biopsy: 1 wk • Arteritic ION without GCA 20% symptoms: • False Negative biopsy 5% 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 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 TREATMENT p.o. Prednisone 80 mg/d 40 mg/d 10 mg/d 1 - 2 weeks 2 - 3 months 1 - 2 years TREATMENT IV Methylprednisolone 1 gm/day for: • bilateral disease • second eye • progressive disease 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 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.