Giant Cell Arteritis presentation for optometrists.pptx

This presentation shows the clinical manifestations of temporal arteritis (giant cell arteritis) in relation to vision and the eye and the way to manage this condition.

1. Giant cell arteritis and the eye How to stop people going completely blind! Peter Simcock FRCP FRCS FRCOphth Consultant Ophthalmic Surgeon West of England Eye Unit, Exeter

2. Only a few eye conditions will make you completely blind in both eyes Gradual Severe glaucoma Severe proliferative diabetic retinopathy Severe inherited eye diseases (retinitis pigmentosa) Sudden Bilateral Anterior Ischaemic Optic Neuropathy from Giant Cell Artertitis

3. What is “completely blind” No useful vision in both eyes Snellen or LogMAR chart vision – useful CF (counting fingers) vision – useful HM (hand movement) vision- useless PL (perception of light) vision – useless NPL (no perception of ) vision - useless

4. What is giant cell arteritis? Also know as temporal arteritis Prevalence is approx. 1 in 160,000 A disease of the elderly and not seen < age 50 Granulomatous inflammation of medium to large arteries Often present with visual symptoms Often associated with polymyalgia rheumatica

5. History 10 th Century Baghdad Ali Ibn Isa al- Kahhal Noted relation between inflamed arteries and loss of sight Suggested removing the temporal arteries to treat and save sight 1890 Jonathan Hutchinson in England Patient with red painful streaks on his head and unable to wear a hat 1932 Bayard Horton First to perform a temporal artery biopsy and show granulomatous vasculitis

6. Epidemiology Negligible incidence if age <50 Average age at presentation is 71 Women 2 to 6 x more affected than men Highest rates in white patients of northern European descent African, Asian and Arab populations have lowest rates Approximately 1/3 of patient with GCA have PMR at presentation

7. Symptoms Visual symptoms Double vision Jaw claudication Myalgias Constitutional symptoms

8. Diagnosis High index of clinical suspicion Elderly patient Head and visual symptoms In 30% of patients with severe permanent visual loss there is antecedent episodes of transient visual disturbance. It is on the differential diagnosis of amaurosis fugax Do blood tests Do Temporal artery biopsy

9. What arteries are involved? Common Superficial temporal artery Central retinal artery Posterior ciliary artery Vertebral artery Less frequent Aorta Coronary arteries

10. Eye Anatomy and its blood supply All come from the Ophthalmic artery, a branch of the internal carotid artery Retina has two circulations Central retinal artery Supplies blood to the inner retina or the nerve connections of the retina Choroidal circulation Supplies blood to the outer retina or photoreceptors and retinal pigment epithelium Rods and cones (photoreceptors) have greatest need of oxygen and glucose of any cell in the body Optic nerve circulation Short posterior ciliary arteries Supply the optic disc and “front portion” of the optic nerve Pial plexus Supplies “back portion” of the optic nerve

11. Sohan Hayreh 1927-2022 Born in a small village in the Punjab Ophthalmology training in London Pioneer and leading authority on vascular diseases of the eye and optic nerve Director of Ocular Vascular Research, University of Iowa

12. The posterior ciliary artery Most affected by GCA Supplies blood to front of optic nerve (optic disc) If blood supply blocked – no blood supply to beginning of optic nerve Results in infarction of the optic disc No nerve connections from the eye to the brain Can result in severe irreversible loss of vision

13. Anterior Ischaemic Optic neuropathy (AION) It describes what it is Anterior – front part of the optic nerve (optic disc) Ischaemic – poor circulation to this portion of the optic nerve Optic neuropathy – a pathology in the optic nerve Two types Non- arteritic (arteriosclerotic involutional blockage) Arteritic (active inflammation induces the blockage and associated with GCA)

14. Non- arteritic Anterior Ischaemic Optic neuropathy (NAION) More frequent than the arteritic variety Patients often arteriopathic (Hypertension, Diabetes, raised lipids) “Disc at risk” Obstructive sleep apnoea syndrome Less severe visual loss than with arteritic variety Often altitudinal visual field loss as sectoral disc ischaemia (compared to complete disc ischaemia with profound visual loss in the arteritic variety)

15. ”Disc at risk”

16. Altitudinal visual field defect

17. Arteritic Anterior Ischaemic Optic neuropathy (AAION) 90% of patients with severe visual loss from GCA Active granulomatous inflammation in the vessel wall More severe blockage of blood flow and infarction of the optic disc Severe visual loss High risk of similar occurrence in fellow eye if left untreated

18. Central retinal artery occlusion 10% of patients with GCA related visual loss Sudden and severe visual loss Attenuated retinal arterial vessels “Cattle trucking” of blood flow Initial retinal whitening due to oedema OCT – inner retina thickening Cherry red spot Chronic – pale disc, white thin vessels, thin retina on OCT

19. Cilioretinal artery occlusion Variation in anatomy of blood supply of retina A congenital anomaly Arise from the short posterior ciliary arteries Supplies blood to some of the macula Can be crucial for preserving vision in a CRAO with central sparing of vision and peripheral loss Can however be affected by GCA with central blurring of vision and peripheral sparing

20. Do not confuse AION with optic neuritis - demyelination of the nerve fibres , not a vascular event Anterior optic neuritis or papillitis Less common, optic disc can be slightly swollen Optic disc then become pale (optic atrophy) Retrobulbar optic neuritis More frequent with demyelination Optic disc looks normal initially then becomes pale (optic atrophy)

21. Disc swelling and its differential diagnosis Bilateral Papilloedema until proved otherwise But don’t forget accelerated hypertension Unilateral Eye or orbital cause AION Papillitis CRVO Optic nerve tumours

22. Posterior ischaemic optic neuropathy (PION) Rare No optic disc swelling Can be arteritic or non arteritic Sometimes a cause of bilateral visual loss after extensive surgery under GA. Hypotension / anaemia thought to be a causative factor especially cardiac or spinal surgery

23. Relative afferent pupil defect Needs fellow eye to be healthy Tells you there is a problem in the optic nerve on that side Does not give you a specific diagnosis but does tell you there is a problem Seen with Arteritic Anterior Ischaemic Optic Neuropathy Non- arteritic Anterior Ischaemic Optic Neuropathy Posterior ischaemic optic neuropathy Papillitis Retrobulbar Optic Neuritis

24. Now need to know some basic anatomy and physiology What is the pupil light reflex? We live in a world where the earth rotates giving us day and night Eye needs to work well in poor light and bright light Retina works best with a limited range of light intensity In poor light, pupil enlarges to let more light in, in bright light it constricts to limit the light falling on the retina Basic reflex arc Autonomic nervous system (do not have to think about it) Sensory / Afferent / Input Central integration (brainstem) Motor / Efferent / Output

25. Reflex arc for the pupil light reflex Afferent / Sensory input Special photoreceptors in retina concerned with this reflex Optic nerve, Chiasm (partial crossover), optic tracts Central integration Midbrain (parasympathetic Eindinger Westphal sub nucleus of III nerve nucleus), also partial crossover of fibres Efferent / Motor output Parasympathetic fibres hitchhike a ride with the III cranial nerve Innervate the sphincter pupillae muscle Increase parasympathetic tone – small pupil Decrease parasympathetic tone – large pupil Forget about the sympathetic to dilator pupillae muscle (Horner’s syndrome)

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27. Rare causes of visual loss in GCA Diplopia from cranial nerve palsy or muscle ischaemia (2 to 15%) III nerve (but exclude Posterior communicating artery aneurysm IV nerve head tilt VI nerve, easy as horizontal diplopia Posterior ischaemic optic neuropathy Cilio-retinal artery occlusion Choroidal infarction Very rarely post-chiasmal visual pathway and cortical visual loss

28. Systemic manifestations of GCA Headache (often temporal) Scalp tenderness when combing hair Jaw claudication with pain when chewing Fever General malaise Aching of muscles (proximal myalgias)

29. Headache in GCA Head pain rather than headache GCA head pain lying down does not feel nice Sore to put head on pillow Migraine head pain lying down in a dark room improves things

30. New classification of GCA Cranial GCA (produces the visual symptoms) AION Jaw claudication Scalp tenderness Large vessel GCA Upper body large arteries (aorta and its major branches) Serous complications such as aortic aneurysms More difficult to diagnose Mixed GCA

31. Polymyalgia rheumatica Frequent reason for elderly patients to be on systemic steroids Symptoms of PMR present in up to 50% of patients with GCA Neck and shoulder pain and stiffness Fatigue Weight loss Fever

32. Temporal artery Tender to palpation and thickened Strength of pulse If poor or no pulse it is occluded Temporal artery ultrasound Helpful for locating artery “Halo” sign signifies inflammation Temporal artery biopsy Crucial to get a good length of artery (up to 2.5cm due to “skip lesions”) Location can be difficult especially if partially occluded with poor pulse Doppler ultrasound assisted location Surgery performed by vascular surgeon Never “too late” to perform a biopsy

33. Laboratory tests and histology Erythrocyte sedimentation rate ESR high > 100mm/ hr C-reactive protein (CRP) elevated > 10mg/L Necrotizing vasculitis Panarteritis with lymphocytes and macrophages Granulomatous inflammation with giant cells Intimal thickening Fragmentation of the internal elastic lamina

34. Treatment - Steroids 80mg to 100mg per day of oral Prednisolone 1 gram of I/V Methyl Prednisolone for 3 days then oral steroid Taper dosage slowly depending on patient symptoms and blood tests Treatment courses typically last 1 to 2 years Important with chronic systemic steroids to check Blood pressure Blood sugar Osteoporosis issues (calcium and vitamin D +/- biphosphonates ) Gastrointestinal disturbances (H2 agonists or proton pump inhibitors)

35. COVID and GCA Slight increase in risk of developing GCA Would avoid COVID jab if has GCA Some patients with bilateral visual loss when initial outbreak occurred in northern Italy

36. What happens if you miss the diagnosis of GCA related visual loss? Second eye can become involved usually within 14 days of first eye involvement and patient them becomes blind Up to 60% of untreated patients progress to bilateral disease Image a 55-year-old professional still working and with no visual or health issues then 2 weeks later HM vision in both eyes and complete loss of independence for the rest of his life

37. Always think of GCA and AION if: Patient over 55 Transient or permanent visual loss in one eye Headache Tenderness of scalp Pain in jaw when eating Diplopia If in doubt – refer urgently If you pontificate – risk of severe bilateral blindness!

38. Quiz on Arteritic AION – true or false? Is caused by atheroma Produces granulomatous inflammation Main involvement is the anterior ciliary arteries Can be diagnosed in patients in their 40’s Always needs an urgent temporal artery biopsy Causes headache that improves with lying down May be seen with severe hypotension associated with major surgery