The Eye in Systemic Diseases

1. The Eye in Systemic Diseases Department of Ophthalmology University Hospitals of Coventry and Warwickshire 2013

2. Learning objectives • Understand which systems may have eye related problems • How the eye problems may herald systemic disease • Understand some of the mechanisms of systemic disease and the eye.

3. Eye- a unique organ • Systemic diseases affect eyes in various ways BUT ALSO • Many of the systemic diseases may be diagnosed first by ophthalmologist

4. Eyes can be affected in: • Endocrine disorders • Disorders of connective tissues • Skin diseases • Inborn errors of metabolism • Gastrointestinal disorders • Infectious diseases • Cardiovascular & Pulmonary diseases • Haemopoietic and lymphoreticular disorders • Neurological and muscular disorders

5. Which common systemic disease is the highest cause of blindness in the working age population?

6. Endocrine disorders: • Most important ones are : • Diabetes Mellitus • Thyroid dysfunction • Pituitary tumours

7. Diabetes mellitus • Most common cause of blindness amongst individuals of working-age ( 20-65 years). • The prevalence of blindness due to DR in Western Communities is estimated as between 1.6-1.9/ 100,000

8. Eye & Diabetes: • Visual loss may occur through • Diabetic retinopathy • Cataract • Glaucoma • Ischaemic Optic Neuropathy • Retinal vein and artery occlusions • IIIrd, IVth and VIth nerve palsies

9. Eye & Diabetes: • Stages of Diabetic Retinopathy • Background • Preproliferative • Proliferative • Maculopathy

10. Pathology of Diabetic Retinopathy • Hyperglycaemia causes- • Basement Membrane thickening • non-enzymatic glycosylation • increased free radical activity • increased flux through the polyol pathway • osmotic damage

11. Microscopic Anatomical Changes Microanerysms • Pericytes which surround the retinal capillaries are damaged • Damage to endothelial cells leads to dilated capillaries and venules • These altered vessels allow serum and blood to leak into the retina

12. Processes of pathology • Ischaemia • Direct effect on Rods and Cones • VEGF release causes new blood vessel formation which bleed and fibrose • Leakage exudative process damages retinal layers

13. Fluorescein angiography Normal vasculature Wide areas of capillary dropout and ischaemia

14. Characterised by Mild Haems / MAs:no more severe than in this standard photo Background / mild non proliferative DR

15. Normal Preproliferative retinopathy Venous beading, IRMA, and Severe/blotch Haem. IRMA Venous loops

16. Cotton Wool Spots • Microinfarcts of nerve fiber layer • Often associated with other preprolif. DR signs • If isolated CWS (no other preprolif DR) may be caused by HTN or recent BS tighter control

17. Proliferative diabetic retinopathy • New vessels grow on the disc (DNV), or elsewhere on the retinal surface (NVE)

18. Proliferative diabetic retinopathy Can evolve very quickly Baseline 6 weeks later

19. Proliferative retinopathy: New Vessels tend to bleed

20. New Vessels Can Also Grow on the Iris • Rubeosis Iridis • Neovascular Glaucoma

21. Late Complications of Proliferative DR Tractional Retinal Detachment

22. Diabetic maculopathy Exudative • Exudates:intraretinal accumulations of lipids leaking from abnormal retinal capillaries and microaneuryisms, may form a circinnate pattern • Leakage of fluid that distorts the retinal architecture Exudative Maculopathy Normal Macula

23. Diabetic maculopathy Ischaemic + Mixed exudative/ischaemic Ischaemia Normal capillary bed

24. Treatment of Diabetic Retinopathy • Scattered laser pan retinal photocoagulation (PRP) for PDR • Focal laser/laser grid for exudative maculopathy • Ischaemic Maculopathy NOT treatable

25. Appearance of fresh laser burns Atrophic, old laser scars Panretinal laser photocoagulation (PRP) for proliferative DR

26. Systemic Risk Factors and DR • Blood sugar and BP control are as good as laser treatment for reducing the risk of retinopathy progression and loss of vision • Nephropathy is a risk factor for DR

27. Hyperlipidaemia and diabetic maculopathy • There is evidence that diabetics who have exudative maculopathy with extensive lipid exudates benefit from active treatment of hyperlipidaemia

28. Endocrine disorders and the eye • Thyroid • Pituitary • Hypothalamus • Parathyroid • Adrenals

29. Pituitary tumours • Headache • Visual field defect • Optic nerve dysfunction • Colour deficit • Visual deterioration • Optic atrophy • MRI scan + Neuro referral

30. Thyroid eye disease (TED) • Patient may be • Euthyroid • Hypothyroid • Hyperthyroid :-40% of patients with Graves disease get eye signs • 4-8% loose vision

31. TED is the commonest cause of proptosis (unilateral or bilateral) in adults

32. Other features: • Lid signs- lid lag, lid retraction • Conjunctival hyperaemia and chemosis • Keratoconjunctivis Sicca • Dysthyroid myopathy • Optic neuropathy

33. Management • Control thyroid status (medical/ surgical) • Lubricants • Orbital decompression surgery • Muscle surgery/ prism in glasses • Lid surgery

34. Other endocrine disorders affecting eyes:

35. Hypertensive retinopathy • Damage to the retina from high blood pressure • Duration increases risk • Worse with • Diabetes • high cholesterol • smoking • Malignant hypertension

36. Symptoms of Hypertensive retinopathy • Grade 1- no symptoms • Grade 2/3- blurring of vision/headache/diplopia • Grade 4- Optic nerve swelling gives blurred vision and field loss. • Patients often have poor renal function and are at risk of stroke and encephalopathy

37. Ophthalmoscopy findings

38. Hypertensive Retinopathy

39. From focal closure of retinal microvasculature/microinfarcts Signs of Hypertensive Retinopathy • CWS (Diast BP often >100mmHg) • Flame shaped Haem. CWS + AV Nicking

40. Arteriolosclerosis often coexist: • -Arteriolar narrowing, focal or diffuse • -Arteriolar colour changes • -AV crossing changes, • e.g. nicking ± flame Haems. • -Vessel sclerosis, threading AV Nicking

41. Hypertensive Retinopathy, Severe • Rarely retinal/macular oedema • Disc oedema + severe macular oedema with macular star in extreme cases (malignant HTN, with BP on the range of 250/150mmHg) • Macular star may develop in weeks & resolve in months • Disc oedema may develop in days and resolve in weeks or months

42. Treatment • Lower blood pressure to below 140/90mmHg

43. Malignant hypertension • Collagen vascular diseases • Renal problems • Eclampsia of Pregnancy • Pheochromocytoma

44. Cardiovascular Diseases causing eye problems • Atrial fibrillation • Aortic stenosis • Hyperlipidaemia- arcus • Hypercholesterolaemia • xanthalasma • Thromboembolism: retinal arteriolar occlusions • Clot • Calcium • cholesterol

45. Retinal Vein Occlusion • Second most common vascular disease causing loss of vision

46. Retinal Vein occlusion • Pathophysiology • thrombus formation • disease of the vein wall • external compression of the vein • Retinal arteries and arterioles and their corresponding veins share a common adventitial sheath. • Atherosclerosis and thickening of the arteriole compresses the vein, eventually causing occlusion.

47. Branch Retinal Vein Occl. Central Retinal Vein Occl. Venous occlusion

48. Etiology • Advancing age - over 50% over 65 yo • 15% under the age of 45 • Hypertension( 64% of patients) • Hyperlipidaemia, diabetes, smoking and obesity. • Raised intraocular pressure. • Inflammatory diseases - sarcoidosis, Behçet's syndrome. • Hyperviscosity states- myeloma. • Thrombophilic disorders (considered in <45 yo • hyperhomocysteinaemia • lupus anticoagulant, • anticardiolipin antibodies; or inherited disorders such as factor V Leiden, protein C or S deficiencies.[3]

49. Connective tissue disorders Marfan’s syndrome: Tall stature Large eyes Myopia Ectopialentis 2°glaucoma, retinal detachment

50. Connective tissue disorders • Ehlers-Danlos syndrome: • Blue sclera, • keratoconus, • ectopia lentis, • angioid streaks