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

• Background / mild non proliferative DR

Characterised by Mild Haems / MAsno more severe
than in this standard photo
15
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
Exudative
Diabetic maculopathy

• Exudatesintraretinal 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
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
Gland Disorder Ocular manifestations
Hypothalamus Suprasellar tumours Optic atrophy, Papilloedema
Parathyroids Hyper/ Hypo-parathyroidism Conjunctival corneal calcification, cataract
Adrenals Pheochromocytoma Addisons disease Cushings disease Hypertensive retinopathy hyperpigmentation Cataract, exophthalmos
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
Signs of Hypertensive Retinopathy

• CWS (Diast BP often gt100mmHg)
• 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
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 lt45 yo
• hyperhomocysteinaemia
• lupus anticoagulant,
• anticardiolipin antibodies or inherited
  disorders such as factor V Leiden, protein C or S
  deficiencies.3

49
Connective tissue disorders

• Marfans syndrome
• Tall stature
• Large eyes
• Myopia
• Ectopia lentis
• 2glaucoma,
• retinal detachment

50
Connective tissue disorders

• Ehlers-Danlos syndrome
• Blue sclera,
• keratoconus,
• ectopia lentis,
• angioid streaks

51

• Acquired connective tissue disorders
• Seropositive
• Rheumatoid arthritis
• Episcleritis
• Scleritis
• Peripheral corneal disease
• Scleromalacia perforans

52
Sero-negative arthritides

• Stills disease,
• Ankylosing spondylitis
• Uveitis
• Cataract
• glaucoma
• Reiters syndrome
• Conjunctivitis, iritis, episcleritis
• retinopathy, optic neuritis

53
(No Transcript)
54
Skin disorders

• Atopic conditions
• Dry skin around lids
• Conjunctivitis
• keratoconus

• Acne rosacea rhinophymoma, keratoconjunctivitis

55
Steven Johnson Syndrome

• Conjunctivitis
• Mucosal ulceration
• Corneal ulceration
• Symblepharon
• Ankyloblepharon
• Uveitis

56
Inborn errors of metabolism

• Homocystinuria
• Ectopia lentis
• retinal detachment
• 2glaucoma

57
Wilsons disease

• Kayser-Fleischer ring
• Sunflower cataract

58
Inborn errors of metabolism

• Galactosaemia
• Cataract
• Refsums disease- (phytanic oxide deficiency)
• Retinitis pigmentosa
• Cataract
• Myopia
• Optic atrophy

59
Gastrointestinal disorders

• Crohns disease
• Ulcerative colitis
• Inflammatory bowel disease
• Vitamin A deficiency
• Abetalipoproteinaemia

Episcleritis and anterior uveitis
Bitots spots, nightblindness, retinitis
pigmentosa
60
Infectious diseases

• Syphilis
• Interstitial keratitis
• Uveitis
• Chorioretinopathy

• Tuberculosis
• Phlycten
• Uveitis
• Choroidal deposits

• Rubella
• Microphthalmia
• Cataract
• Glaucoma
• Uveitis
• Salt pepper retinopathy

• Others
• Viral infections Varicella-Zoster
• Parasitic Toxoplasmosis
• Granulomatous Sarcoid
• Fungal

61
Blood lymphoreticular disorders cause
retinopathy

• Anaemias, leukaemias
• Sickle cell disease
• Lymphomas
• Polycythaemia

62
Neurological Muscular disorders

• Multiple sclerosis
• Optic neuritis,
• eye movement disorders
• Neurofibromatosis
• Proptosis, lid lesions, glaucoma, optic nerve
  gliomas/ meningiomas
• Myasthenia gravis
• Ptosis, diplopia, ocular motility disorders

63
What have we discussed

• Most systems in the body will have an eye problem
  association
• Eyes may indicate systemic disease
• Failure to refer patients with systemic diseases
  for eye examination could result in blindness.