9/11/11. Temporal Arteritis. Background. Background. Richard E. Castillo, OD, DO NORTHEASTERN STATE UNIVERSITY Director, Ophthalmic Surgery Service

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1 Temporal Arteritis Richard E. Castillo, OD, DO NORTHEASTERN STATE UNIVERSITY Director, Ophthalmic Surgery Service 1 Background Giant Cell Arteritis Temporal Arteritis Cranial Arteritis Granulomatous Arteritis 2 Background A systemic inflammatory vasculitis Etiology unknown Affects medium-large vessels of the head and neck 3 1

2 Background A disease of the elderly Wide variety of systemic, neurological, and ocular complications 4 Background Visual loss is one of the most significant causes of morbidity in GCA Permanent visual impairment in up to 60% of patients 5 Background GCA is a Clinical Diagnosis GCA is a true neuroophthalmic emergency 6 2

Etiology: Unknown? Genetic Familial aggregation in northern europeans?

Autoimmune Cell mediated immune reaction directed at antigens in or near

Inflammatory infiltrate surrounding a fragmented internal elastic lamina

Likely dysfunction of cellular immunity 8 Pathophysiology: GCA Inflammation of

3 Etiology: Unknown? Genetic Familial aggregation in northern europeans? Infectious Chlamydia and parvovirus? Autoimmune Cell mediated immune reaction directed at antigens in or near elastic tissue in arterial walls 7 Pathophysiology: GCA Histopathologically Inflammatory infiltrate surrounding a fragmented internal elastic lamina within the media of the arterial wall Infiltrate Mononuclear cells Giant cells Likely dysfunction of cellular immunity 8 Pathophysiology: GCA Inflammation of the aortic arch and its branches Common carotid a. Inominate a. Vertebral a. Arteries involved in segmental manner Internal elastic lamina 9 3

Pathophysiology: GCA Most commonly Extracranial vertebral a. Superficial temporal a. Posterior ciliary a. Ophthalmic a. Less commonly Internal & external carotid a. Central retinal a.

Intracranial arteries are rarely involved 11 Diagnostic Criteria Traditional (sensitivity, 93.5%; specificity, 91.2%; 3 0f 5 criteria must be met) 1. Age of onset of 50 years or older 2.

4 Pathophysiology: GCA Most commonly Extracranial vertebral a. Superficial temporal a. Posterior ciliary a. Ophthalmic a. Less commonly Internal & external carotid a. Central retinal a. 10 Pathophysiology: GCA Postmortem studies Proximal & distal aorta Subclavian a. Brachial a. Abdominal a. Intracranial arteries are rarely involved 11 Diagnostic Criteria Traditional (sensitivity, 93.5%; specificity, 91.2%; 3 0f 5 criteria must be met) 1. Age of onset of 50 years or older 2. Onset of new headache 3. Temporal artery tenderness or reduced pulsation 4. Elevated Westergren erythrocyte sedimentation rate 5. Elevated c-reactive Protein 6. Abnormal arterial biopsy American College of Rheumatology Criteria for Classification of Giant cell arteritis. 12 4

Diagnostic Criteria Alternative (sensitivity, 95.3%; specificity, 90.7%; 3 of 6 criteria must be met) 1. Age at onset of 50 years or older 2. Onset of new headache 3.

5 Diagnostic Criteria Alternative (sensitivity, 95.3%; specificity, 90.7%; 3 of 6 criteria must be met) 1. Age at onset of 50 years or older 2. Onset of new headache 3. Temporal artery tenderness or reduced pulsation 4. Claudication of jaw 5. Scalp tenderness or nodules 6. Abnormal artery biopsy American College of Rheumatology Criteria for Classification of Giant cell arteritis. 13 Incidence/Risk: GCA In the U.S cases/100,000 Incidence peaks in eighth decade Age 50+ (most important risk factor) Incidence is higher in northern U.S. Higher in caucasians of European descent Females 2-4X more likely than male 14 Morbidity: GCA Mild GCA Generalized myalgias & fatigue May mimic Polymyalgia rheumatica? Intermittent claudication: 50% Jaws Tongue Throat 15 5

Mortality/Morbidity: GCA Nervous system alterations: 30%

Symptoms: 33% - 50% overall 40%-50% transient Amaurosis fugax

Unilateral: 50% Bilateral: 50% 17 Morbidity: GCA Rarely weakening

dilatation Aortic regurgitation: 33% Aortic arch syndrome: 9-14%

6 Mortality/Morbidity: GCA Nervous system alterations: 30% Mono/poly-neuropathy: 14% TIA/CVA: 7% 16 Morbidity: GCA Visual Symptoms: 33% - 50% overall 40%-50% transient Amaurosis fugax Diplopia 50%-60% permanent visual loss Partial or complete Unilateral: 50% Bilateral: 50% 17 Morbidity: GCA Rarely weakening of aortic wall Aneurism formation: 6 years Aortic annular dilatation Aortic regurgitation: 33% Aortic arch syndrome: 9-14% Decreased UE pulses/bp Arm/leg claudication Raynauds phenomenon TIA Coronary ischemia/abdominal angina 18 6

Mortality: GCA Involvement of major vessels (aorta) increases risk of death 50%

History of Illness Onset may be abrupt or insidious Symptoms usually present

Fatigue Weight loss 20 Systemic History GCA may begin with complaints of:

7 Mortality: GCA Involvement of major vessels (aorta) increases risk of death 50% of those with thoracic aortic aneurism died suddenly from aortic dissection 19 History of Illness Onset may be abrupt or insidious Symptoms usually present weeks-months prior to diagnosis Constitutional symptoms present in most Anorexia Fatigue Weight loss 20 Systemic History GCA may begin with complaints of: New-onset localized headache (hallmark) Anorexia Fever Malaise Myalgia Night sweat Weight loss 21 7

22 Ophthalmic Manifestations AION (posterior ciliary arteries) Chalky white edematous disc (w/ or w/o splinter hemorrhages)

8 Ophthalmic History 50% eventually experience visual symptoms Transient visual blurring Usually reversible Diplopia Eye pain Sudden loss of vision Almost always permanent Usually AION Vision loss, partial or complete variably reported to be 10-60% 22 Ophthalmic Manifestations AION (posterior ciliary arteries) Chalky white edematous disc (w/ or w/o splinter hemorrhages) PION CRAO BRAO Choroidal ischemia 23 Neuro-ophthalmic manifestations Diplopia Ptosis Nystagmus INO Pupillary abnormalities 24 8

Manifestations Atrophy of temporal and tongue muscles Decreased temporal artery blood flow Cerebrovascular disease in 1-25% is most

9 Systemic Manifestations New-onset localized HA Temporal or occipital Scalp tenderness Temporal tenderness Visibly enlarged, pulseless temporal artery Jaw claudication Facial pain/earache Toothache Tongue/palate pain Odynophagia Bruits 25 Systemic Manifestations Atrophy of temporal and tongue muscles Decreased temporal artery blood flow Cerebrovascular disease in 1-25% is most common cause of death 26 Neurologic Manifestations Myopathy Neuro-otologic syndromes Neuropsychiatric syndromes Peripheral neuropathies Seizures 27 9

with prominent vascular involvement Neoplasms Arteriosclerotic vascular disease Arteriovenous

10 Other manifestations Cardiovascular Pulmonary Gastrointestinal Renal Dermatologic 28 Other considerations Transient ischemic attack Polymyalgia rheumatica Systemic infections Amyloidosis with prominent vascular involvement Neoplasms Arteriosclerotic vascular disease Arteriovenous fistulas Other forms of vasculitis 29 Lab Studies ESR CRP Fibrinogen CBC Hepatic enzymes Immune function tests 30 10

Imaging Studies: GCA Arteriography CT/MRI Color Duplex

Look for halo of edema around vessel wall 31 Visual

nasal sectorial defect Central scotoma 32 Diagnostic

Performed on most symptomatic side May require repeat

11 Imaging Studies: GCA Arteriography CT/MRI Color Duplex US Valid alternative to TAB? Look for halo of edema around vessel wall 31 Visual Field Testing Inferior altitudinal defect Inferior nasal sectorial defect Central scotoma 32 Diagnostic Procedures: GCA Superficial temporal artery biopsy Performed on most symptomatic side May require repeat on other side Therapy should not be withheld pending the performance and results of TAB

Medical Care: GCA High Dose Steroids Standard of care Goals: reverse

disease activity suppressed Switch to orals & taper off to maintenance

Follow ESR, constitutional & vascular symptoms 34 Medical Care In

Cyclosporin-methotrexate May also use above in patients requiring

12 Medical Care: GCA High Dose Steroids Standard of care Goals: reverse disease prevent progression Initial dose of 1-2 mg/kg/d IV until disease activity suppressed Switch to orals & taper off to maintenance dose for ~ 2 years. Follow ESR, constitutional & vascular symptoms 34 Medical Care In steroid-resistant cases Cyclosporine-azathioprine Cyclosporin-methotrexate May also use above in patients requiring protracted treatment 35 Inpatient Care Monitor daily symptoms visual acuity ESR/CRP Regular follow-up after successful initial management is standard 36 12

37 Consultations Rheumatologist When biopsy is negative but clinical suspicion remains high enough to

13 Surgical Care Aside from TAB there is none for GCA. Up to 41% of those with thoracic aorta aneurism may require surgery. 37 Consultations Rheumatologist When biopsy is negative but clinical suspicion remains high enough to warrant high dose steroids When other forms of immunosuppresive therapy are considered 38 Complications CVA Aortic artery aneurisms Thoracic aorta Ascending aorta Myocradial infarction Visceral ischemia 39 13

14 Prognosis GCA is a chronic disease that may last for years Overall course Progressive improvement Eventual resolution Course is highly variable and in some may be protracted for months to years 40 Medical/Legal Pitfalls Failure to consider GCA, or TIA in an elderly patient with amaurosis fugax Failure to consider polymyalgia rheumatica 41 Polymyalgia rheumatica Diagnosis of exclusion GCA Rheumatoid arthritis Polymyositis Chronic infection Malignant neoplasm Aching & morning stiffness lasting 1/2 hour or more in 2-3 commonly affected areas Neck Shoulder girdle Hip girdle ESR elevation with rapid response to small doses of steroid 42 14

Preventing blindness: Ultrasound in Giant cell arteritis

Preventing blindness: Ultrasound in Giant cell arteritis Elizabeth Jernberg, MD Associate Clinical Professor of Medicine Division of Rheumatology University of Washington Virginia Mason Medical Center