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Ocular Manifestations Of
Systemic Diseases
INTRODUCTION
 The eyes are frequently involved in diseases
Affecting the rest of the body.
 Ocular manifestations in certain multisystem
Disorders may offer a diagnostic clue.
Eye examination
 Vision
 External – Look at eyelids, make sure everything looks normal.
 Pupils
 Relative afferent papillary defect
 See if pupils are reacting normally
 Motility – Have the patient look up, down, right and left.
 Exam of anterior segment
 Dilated ophthalmoscopy – Examine retina, optic nerve, and vessels.
 Visual fields – Place you finger in different places in patient’s visual field and
ask him if he sees it. Test one eye at a time.
INVOLVED SYSTEMIC DISEASES
1. Connective tissue diseases
2. Spondyloarthropathies
3. Digestive-inflammatory bowel
disease
4. Multisystem non-infectious
diseases
5. Infectious disease
6. Mucocutaneous disease
7. Cardiovascular disease
8. Endocrine-metabolic disease
9. Myopathies
10. Neurologic diseases
11. Haematological disease
Connective Tissue Diseases
A. Rheumatoid arthritis
 Keratoconjunctivitis sicca
 Episcleritis
 Scleritis
 Peripheral ulcerative keratitis
 Uveitis
 Rheumatoid Episcleritis  Rheumatoid Scleromalacia Perforans
 Rheumatoid Corneal UlcerationRheumatoid Scleritis
Juvenile Idiopathic Arthritis
 Idiopathic Arthritis in
patients younger than 16
years and lasts at least 6
weeks.
 Classification
Oligo or pauciarticular
Polyarticular RF + /
RF-
Psoriatic
Enthesitis
 Risk factor for uveitis
Early onset
female
ANA +
oligoarticular
HLA-DR5
Juvenile Idiopathic Arthritis
Ocular feature-
1. AU- chronic and granulomatous
2. Posterior synechia
3. Band keratopathy
4. Cataract
5. Glaucoma
C. Systemic Lupus Erythematosus (Lupus)
Ocular feature
Dry eyes
 Scleritis
Peripheral corneal ulcers
 Retinopathy
Optic neuropathy
D. Wegener Granulomatosis
E. Sjogren Syndrome
Sjogren Syndrome
F. Giant Cell Arteritis
G. Marfan syndrome
2. Spondyloarthropathies
A. Ankylosing spondylitis
B. Reiter syndrome/Reactive arthritis
Keratoderma blennorrhagica in reactive arthritis
3. Inflammatory Bowel Disease
a) Crohn’s disease
b)Ulcerative colitis
AAU
Dry eye
Conjunctivitis
Scleritis
Episcleritis
4. Multisystem noninfectious diseases
A. Sarcoidosis  Respiratory symptoms
 Skin lesion
 Lympadenopathy
 Cardiac disease
 Neurologic disease
Sarcoidosis
Ocular feature  Granulomatous anterior uveitis
 Mutton fat KP
 Iris nodules (koeppe / Busaca)
 TM nodules
 Vitrious opacities (snow ball)
 Periphlebitis ( candle wax drippings)
 Optic disc nodules
 Dry eye
 Bilaterality
Very large granulomatous ‘mutton fat’ keratic precipitates
Sarcoidosis
granulomata and periphlebitis
‘Candle wax drippings’snowballs
Lacrimal gland enlargement in sarcoidosis
B. Behçet disease
 Criteria for Behçet's disease:
Mouth sores (oral ulcers) at least 3 times in 12 months
Any 2 of the following:
Recurring genital sores/ulcers
Uveitis
Skin: Pustules or erythema nodosum
Positive pathergy (skin prick test)
Major aphthous ulceration superficial thrombophlebitis
Behçet disease
 Ocular feature  AAU
 Vitritis
 Retinitis
 Retinal vasculitis
 Optic disc oedama
 Optic atrophy
Hypopyon in a white eye
occlusive vasculitis
C. Vogt Koyanagi Harada syndrome
Vitiligo and poliosis in Vogt–Koyanagi–Harada
syndrome ‘Sunset glow’ fundus
5. Infectious disease
A. Tuberculosis
 ocular feature
 Anterior uveitis – granulomatous
 Vitritis
 Choroidal granuloma
 Choroiditis
 Retnal vasculitis
 Lupus vulgaris
Choroidal granuloma
Occlusive tuberculous periphlebitis. -Superior retinal branch
occlusion
B. Syndrome acquired immunodeficiency (SAID)
Kaposi sarcoma HIV wasting syndrome HIV microangiopathy
C. Syphilis
 Ocular features  Anterior uveitis
 Chorioretinitis
 Acute syphilitic posterior placoid
chorioretinopathy (ASPPC)
 Retinitis
 Optic neuritis and neuroretinitis
 Argyll Robertson pupils
Maculopapular rash in
secondary disease;
old multifocal chorioretinitis
acute posterior placoid chorioretinitis
D. Toxoplasmosis
 Ocular features  ‘Spill-over’ anterior uveitis
 single inflammatory focus of fluffy
white retinitis or retinochoroiditis
(‘satellite lesion’)
 Vitritis
 Vasculitis
 Optic disc oedema
 Visual loss
Typical ‘satellite’
lesion adjacent to an old scar
severe vitreous haze and
‘headlight in the fog’
appearance of lesion
Macular involvement
6. Mucocutaneous disease
A. Pemphigoid
syndromes
 Ocular feature
 Bilateral conjunctivitis
 Symblepharon
 Dry eye
 Trichiasis
 Ankyloblepharon
 Corneal opacities
moderate fibrosis with forniceal
shortening and symblepharon formation
ankyloblepharon
severe skin blistering
B. Stevens-Johnson syndrome
Haemorrhagic lid crusting severe acute conjunctivitis pseudomembrane
Corneal keratinization extensive purpuric lesions Haemorrhagic lip crusting
7. Cardiovascular disease
 Grade 1. Mild generalized retinal arteriolar narrowing
 Grade 2. Focal arteriolar narrowing and arteriovenous nipping
(copper wiring)
 Grade 3. Grade 2 plus retinal haemorrhages (dot, blot, flame), hard
exudates (‘macular star’) and cotton wool spots.
 Grade 4. Severe grade 3 plus optic disc swelling.this is a marker of
malignant hypertension.
Hypertensive retinopaty
Generalized arteriolar attenuation focal arteriolar attenuation red-free photograph showing
arteriovenous nipping
copper wiring grade 3 retinopathy with macular star grade 4 hypertensive retinopathy
B.Carotid stenosis
8. Endocrine-metabolic disease
A. Diabetes mellitus
Microaneurysms Dot & blot VS splinter hemorrhage Hard exudate
Diabetic retinopathy
Hard Exudate VS Cotton Wool Spot
Venous beading
Intra-retinal microvascular abnormalities (IRMA)
Clinical Significant Macular Edema
(CSME)
1 of 3
Retinal edema
within 500 microns
of centre fovea
Hard exudates within
500 microns of fovea
if ass with adjacent
retinal thickening
Retinal edema > 1 disc
diameter, any part is
within 1 disc diameter
of centre of fovea
Diabetic retinopathy
Vitreous hemorrhage
Tractional retinal detachment
Rubeosis iridis
(neovascularisation of the iris)
Neovascular glaucoma
B. Thyroid eye disease
 CLINICAL MANIFESTATION
 5 main clinical manifestations of TED are:
I. Soft tissue involvement (periorbital & lid swelling, conjuctival
hyperemia).
II. Lid retraction
III. Proptosis (passive or mechanical protrusion of eye ball)
IV. Optic neuropathy (serious complication – compression of optic nerve may
lead to visual impairement)
V. Restrictive myopathy (ocular motilty is reduced initially by inflammatory
edema & later by fibrosis)
Thyroid eye disease
moderate bilateral symmetrical lid retraction –Dalrymple sign severe bilateral lid retraction – Kocher sign
right lid lag on downgaze – von Graefe sign Proptosis in thyroid eye disease
9. Myopathies
 A. Myasthenia gravis
10. Neurologic Diseases
A. Multiple Sclerosis
B. Neurofibromatosis
Nodular plexiform
neurofibroma of the eyelid
axial CT image
showing right proptosis
with fusiform
enlargement of the
optic nerve due to
glioma;
right proptosis due to optic nerve
glioma
Lisch nodules
11. Haematological Disease
Leukemia
 Ocular feature
 Retinal haemorrhages and cotton
wool spots
 Roth spots
 Peripheral retinal
neovascularization
 Retinal and choroidal infiltrates
 Optic nerve infiltration
 Visual loss
 Orbital involvement
 Iris thickening, iritis and
pseudohypopyon
Leukemia
Retinal haemorrhages in
leukaemia. - Numerous
flame haemorrhages with cotton
wool spots and Roth
spots
large retrohyaloid
haemorrhage – a separate white cell
layer is evident
Roth spots
Ocular Manifestations Of Systemic Diseases

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Ocular Manifestations Of Systemic Diseases

  • 2. INTRODUCTION  The eyes are frequently involved in diseases Affecting the rest of the body.  Ocular manifestations in certain multisystem Disorders may offer a diagnostic clue.
  • 3. Eye examination  Vision  External – Look at eyelids, make sure everything looks normal.  Pupils  Relative afferent papillary defect  See if pupils are reacting normally  Motility – Have the patient look up, down, right and left.  Exam of anterior segment  Dilated ophthalmoscopy – Examine retina, optic nerve, and vessels.  Visual fields – Place you finger in different places in patient’s visual field and ask him if he sees it. Test one eye at a time.
  • 4. INVOLVED SYSTEMIC DISEASES 1. Connective tissue diseases 2. Spondyloarthropathies 3. Digestive-inflammatory bowel disease 4. Multisystem non-infectious diseases 5. Infectious disease 6. Mucocutaneous disease 7. Cardiovascular disease 8. Endocrine-metabolic disease 9. Myopathies 10. Neurologic diseases 11. Haematological disease
  • 5. Connective Tissue Diseases A. Rheumatoid arthritis  Keratoconjunctivitis sicca  Episcleritis  Scleritis  Peripheral ulcerative keratitis  Uveitis
  • 6.  Rheumatoid Episcleritis  Rheumatoid Scleromalacia Perforans  Rheumatoid Corneal UlcerationRheumatoid Scleritis
  • 7. Juvenile Idiopathic Arthritis  Idiopathic Arthritis in patients younger than 16 years and lasts at least 6 weeks.  Classification Oligo or pauciarticular Polyarticular RF + / RF- Psoriatic Enthesitis  Risk factor for uveitis Early onset female ANA + oligoarticular HLA-DR5
  • 8. Juvenile Idiopathic Arthritis Ocular feature- 1. AU- chronic and granulomatous 2. Posterior synechia 3. Band keratopathy 4. Cataract 5. Glaucoma
  • 9. C. Systemic Lupus Erythematosus (Lupus) Ocular feature Dry eyes  Scleritis Peripheral corneal ulcers  Retinopathy Optic neuropathy
  • 13. F. Giant Cell Arteritis
  • 16. B. Reiter syndrome/Reactive arthritis Keratoderma blennorrhagica in reactive arthritis
  • 17. 3. Inflammatory Bowel Disease a) Crohn’s disease b)Ulcerative colitis AAU Dry eye Conjunctivitis Scleritis Episcleritis
  • 18. 4. Multisystem noninfectious diseases A. Sarcoidosis  Respiratory symptoms  Skin lesion  Lympadenopathy  Cardiac disease  Neurologic disease
  • 19. Sarcoidosis Ocular feature  Granulomatous anterior uveitis  Mutton fat KP  Iris nodules (koeppe / Busaca)  TM nodules  Vitrious opacities (snow ball)  Periphlebitis ( candle wax drippings)  Optic disc nodules  Dry eye  Bilaterality Very large granulomatous ‘mutton fat’ keratic precipitates
  • 20. Sarcoidosis granulomata and periphlebitis ‘Candle wax drippings’snowballs Lacrimal gland enlargement in sarcoidosis
  • 21. B. Behçet disease  Criteria for Behçet's disease: Mouth sores (oral ulcers) at least 3 times in 12 months Any 2 of the following: Recurring genital sores/ulcers Uveitis Skin: Pustules or erythema nodosum Positive pathergy (skin prick test) Major aphthous ulceration superficial thrombophlebitis
  • 22. Behçet disease  Ocular feature  AAU  Vitritis  Retinitis  Retinal vasculitis  Optic disc oedama  Optic atrophy Hypopyon in a white eye occlusive vasculitis
  • 23. C. Vogt Koyanagi Harada syndrome Vitiligo and poliosis in Vogt–Koyanagi–Harada syndrome ‘Sunset glow’ fundus
  • 24. 5. Infectious disease A. Tuberculosis  ocular feature  Anterior uveitis – granulomatous  Vitritis  Choroidal granuloma  Choroiditis  Retnal vasculitis  Lupus vulgaris Choroidal granuloma Occlusive tuberculous periphlebitis. -Superior retinal branch occlusion
  • 25. B. Syndrome acquired immunodeficiency (SAID) Kaposi sarcoma HIV wasting syndrome HIV microangiopathy
  • 26. C. Syphilis  Ocular features  Anterior uveitis  Chorioretinitis  Acute syphilitic posterior placoid chorioretinopathy (ASPPC)  Retinitis  Optic neuritis and neuroretinitis  Argyll Robertson pupils Maculopapular rash in secondary disease; old multifocal chorioretinitis acute posterior placoid chorioretinitis
  • 27. D. Toxoplasmosis  Ocular features  ‘Spill-over’ anterior uveitis  single inflammatory focus of fluffy white retinitis or retinochoroiditis (‘satellite lesion’)  Vitritis  Vasculitis  Optic disc oedema  Visual loss Typical ‘satellite’ lesion adjacent to an old scar severe vitreous haze and ‘headlight in the fog’ appearance of lesion Macular involvement
  • 28. 6. Mucocutaneous disease A. Pemphigoid syndromes  Ocular feature  Bilateral conjunctivitis  Symblepharon  Dry eye  Trichiasis  Ankyloblepharon  Corneal opacities moderate fibrosis with forniceal shortening and symblepharon formation ankyloblepharon severe skin blistering
  • 29. B. Stevens-Johnson syndrome Haemorrhagic lid crusting severe acute conjunctivitis pseudomembrane Corneal keratinization extensive purpuric lesions Haemorrhagic lip crusting
  • 30. 7. Cardiovascular disease  Grade 1. Mild generalized retinal arteriolar narrowing  Grade 2. Focal arteriolar narrowing and arteriovenous nipping (copper wiring)  Grade 3. Grade 2 plus retinal haemorrhages (dot, blot, flame), hard exudates (‘macular star’) and cotton wool spots.  Grade 4. Severe grade 3 plus optic disc swelling.this is a marker of malignant hypertension.
  • 31. Hypertensive retinopaty Generalized arteriolar attenuation focal arteriolar attenuation red-free photograph showing arteriovenous nipping copper wiring grade 3 retinopathy with macular star grade 4 hypertensive retinopathy
  • 33. 8. Endocrine-metabolic disease A. Diabetes mellitus Microaneurysms Dot & blot VS splinter hemorrhage Hard exudate
  • 34. Diabetic retinopathy Hard Exudate VS Cotton Wool Spot Venous beading Intra-retinal microvascular abnormalities (IRMA)
  • 35. Clinical Significant Macular Edema (CSME) 1 of 3 Retinal edema within 500 microns of centre fovea Hard exudates within 500 microns of fovea if ass with adjacent retinal thickening Retinal edema > 1 disc diameter, any part is within 1 disc diameter of centre of fovea
  • 37. Rubeosis iridis (neovascularisation of the iris) Neovascular glaucoma
  • 38. B. Thyroid eye disease  CLINICAL MANIFESTATION  5 main clinical manifestations of TED are: I. Soft tissue involvement (periorbital & lid swelling, conjuctival hyperemia). II. Lid retraction III. Proptosis (passive or mechanical protrusion of eye ball) IV. Optic neuropathy (serious complication – compression of optic nerve may lead to visual impairement) V. Restrictive myopathy (ocular motilty is reduced initially by inflammatory edema & later by fibrosis)
  • 39. Thyroid eye disease moderate bilateral symmetrical lid retraction –Dalrymple sign severe bilateral lid retraction – Kocher sign right lid lag on downgaze – von Graefe sign Proptosis in thyroid eye disease
  • 40. 9. Myopathies  A. Myasthenia gravis
  • 41. 10. Neurologic Diseases A. Multiple Sclerosis
  • 42. B. Neurofibromatosis Nodular plexiform neurofibroma of the eyelid axial CT image showing right proptosis with fusiform enlargement of the optic nerve due to glioma; right proptosis due to optic nerve glioma Lisch nodules
  • 43. 11. Haematological Disease Leukemia  Ocular feature  Retinal haemorrhages and cotton wool spots  Roth spots  Peripheral retinal neovascularization  Retinal and choroidal infiltrates  Optic nerve infiltration  Visual loss  Orbital involvement  Iris thickening, iritis and pseudohypopyon
  • 44. Leukemia Retinal haemorrhages in leukaemia. - Numerous flame haemorrhages with cotton wool spots and Roth spots large retrohyaloid haemorrhage – a separate white cell layer is evident