Ocular manifestations of systemic disease

Ocular Manifestations Ocular Manifestations of Systemic Disease of Systemic Disease

Dr. Riyad G. BanayotDr. Riyad G. Banayot

OCULAR OCULAR MANIFESTATIONSMANIFESTATIONS

SYSTEMIC DISEASESYSTEMIC DISEASE

Categories of systemic diseaseCategories of systemic disease

CongenitalCongenital VascularVascular Endocrine/MetabolicEndocrine/Metabolic AutoimmuneAutoimmune IdiopathicIdiopathic

InfectiousInfectious Drugs/ToxinsDrugs/Toxins NeoplasticNeoplastic TraumaTrauma

Eye examinationEye examination

VisionVision External – Look at eyelids, make sure everything looks normal.External – Look at eyelids, make sure everything looks normal. PupilsPupils

Relative afferent papillary defectRelative afferent papillary defect See if pupils are reacting normallySee if pupils are reacting normally

Motility – Have the patient look up, down, right and left.Motility – Have the patient look up, down, right and left. Exam of anterior segment Exam of anterior segment Dilated ophthalmoscopy – Examine retina, optic nerve, and Dilated ophthalmoscopy – Examine retina, optic nerve, and

vessels.vessels. Visual fields – Place you finger in different places in patientVisual fields – Place you finger in different places in patient’’s s

visual field and ask him if he sees it. Test one eye at a time.visual field and ask him if he sees it. Test one eye at a time.

CongenitalCongenital disorders with ocular disorders with ocular manifestationsmanifestations

Down SyndromeDown Syndrome Marfan's SyndromeMarfan's Syndrome Myotonic DystrophyMyotonic Dystrophy Tuberous SclerosisTuberous Sclerosis Congenital Metabolic DisordersCongenital Metabolic Disorders

Lysosomal storageLysosomal storage Carbohydrate metabolismCarbohydrate metabolism

NeurofibromatosisNeurofibromatosis

MarfanMarfan’’s Syndromes Syndrome

Myotonic DystrophyMyotonic Dystrophy

NeurofibromatosisNeurofibromatosis

VascularVascular disorders with ocular disorders with ocular manifestationsmanifestations

HypertensionHypertension MigrainesMigraines EmboliEmboli Amaurosis fugaxAmaurosis fugax

Vascular HypertensionVascular Hypertension

Affects eyes the same way it affects the rest of Affects eyes the same way it affects the rest of the bodythe body

– – Arteriolar sclerosisArteriolar sclerosis

– – Direct pressure damageDirect pressure damage Number one predisposing factor for otherNumber one predisposing factor for other

eye disorders like CRVO, BRVOeye disorders like CRVO, BRVO

Vascular HypertensionVascular Hypertension

In a normal eye, the vessel walls areIn a normal eye, the vessel walls are

transparent - you see the blood (red)transparent - you see the blood (red) Arteriolar sclerosis causing thickening of the Arteriolar sclerosis causing thickening of the

vessel wallsvessel walls

– – Early sclerosis - Early sclerosis - ““copper wirecopper wire”” vessel vessel appearanceappearance

– – More sclerosis - More sclerosis - ““silver wiresilver wire”” appearance appearance

Vascular HypertensionVascular Hypertension

Arteries and veins share the same sheathArteries and veins share the same sheath As arteriolar walls sclerose and thicken, the vein As arteriolar walls sclerose and thicken, the vein

is compressed - AV Nicking is compressed - AV Nicking

Vascular HypertensionVascular Hypertension

Acutely elevated blood pressure can lead to Acutely elevated blood pressure can lead to fibrinoid necrosis of blood vessel wallsfibrinoid necrosis of blood vessel walls

– – Get exudates, cotton wool spots, Get exudates, cotton wool spots, hemorrhageshemorrhages

– – Can also see macular edema, nerve Can also see macular edema, nerve edemaedema

Hypertensive RetinopathyHypertensive Retinopathy

Hypertensive RetinopathyHypertensive Retinopathy

Hypertensive RetinopathyHypertensive Retinopathy

Vascular HypertensionVascular Hypertension

Classically, it is taught that hypertensivesClassically, it is taught that hypertensives

should be referred for ophthalmologicshould be referred for ophthalmologic

evaluationevaluation

– – No point in this - we canNo point in this - we can’’t do anythingt do anything

– – Send them to their primary care physician for Send them to their primary care physician for BP controlBP control

– – To the emergency room if systolic BP over To the emergency room if systolic BP over 200.200.

Vascular MigrainesVascular Migraines

Migraines are a vasospastic phenomenonMigraines are a vasospastic phenomenon Usually the effects are temporary, but theyUsually the effects are temporary, but they

can be permanent - like a stroke can be permanent - like a stroke

Vascular MigrainesVascular Migraines

Ocular manifestationsOcular manifestations

– – ScintillationsScintillations

– – Transient homonymous hemifield lossTransient homonymous hemifield loss

– – Transient cortical visual lossTransient cortical visual loss

– – Amaurosis fugaxAmaurosis fugax

– – Rarely, CRAO or BRAORarely, CRAO or BRAO

Vascular MigrainesVascular Migraines

TreatmentTreatment

– – Prevent frequent attacksPrevent frequent attacks

– – Examine for visual lossExamine for visual loss

– – Consider discontinuation of oral Consider discontinuation of oral contraceptives as they can lead to thrombotic contraceptives as they can lead to thrombotic eventsevents

Vascular EmboliVascular Emboli

Usually are atherosclerotic/cholesterolUsually are atherosclerotic/cholesterol

plaques from the carotidsplaques from the carotids Can be from other sourcesCan be from other sources

– – heart and heart valvesheart and heart valves

– – IV drug abuse - talcIV drug abuse - talc Manifests as acute visual loss such asManifests as acute visual loss such as

CRAO, BRAOCRAO, BRAO

Emboli affecting the eyesEmboli affecting the eyes

Cholesterol EmbolusCholesterol Embolus

Talc EmboliTalc Emboli

Vascular Amaurosis fugaxVascular Amaurosis fugax

Sudden, painless monocular loss of visionSudden, painless monocular loss of vision

– – Usually manifests like Usually manifests like ““a window shadea window shade

coming downcoming down”” or dimming or dimming

– – By definition, vision returns to normal within By definition, vision returns to normal within a few minutesa few minutes

Caused by temporary arterial occlusionCaused by temporary arterial occlusion

Vascular Blood DyscrasiasVascular Blood Dyscrasias

Hyperviscosity syndromes - thrombosisHyperviscosity syndromes - thrombosis

– – Including polycythemia, myeloma, Including polycythemia, myeloma, leukemialeukemia Thrombocytopenia - bleedingThrombocytopenia - bleeding Anemia - ischemiaAnemia - ischemia

– – Including sickle cell anemiaIncluding sickle cell anemia Lymphoma Lymphoma

Hyperviscosity effects on retinaHyperviscosity effects on retina

LeukemiaLeukemia

LeukemiaLeukemia

Sickle Cell RetinopathySickle Cell Retinopathy

Sickle Cell RetinopathySickle Cell Retinopathy

Endocrine/MetabolicEndocrine/Metabolic

Primary disease in this category is:Primary disease in this category is:

Diabetes MellitusDiabetes Mellitus

EndocrineEndocrineDiabetes MellitusDiabetes Mellitus

Most common cause of blindness in theMost common cause of blindness in the

U.S. between the ages of 18 and 74U.S. between the ages of 18 and 74 8000 people become blind annually in U.S.8000 people become blind annually in U.S. Many effects on the eyeMany effects on the eye

- Changes refraction- Changes refraction

- Causes cataracts- Causes cataracts

- Most important is retinopathy- Most important is retinopathy

EndocrineEndocrineDiabetes MellitusDiabetes Mellitus

Ocular problems are a function of durationOcular problems are a function of duration

of diseaseof disease

- 5 years - 23%- 5 years - 23%

- 20 years - 90%- 20 years - 90%

- 30 years - 99%- 30 years - 99%

- Rare in children under 10 regardless of - Rare in children under 10 regardless of duration of diseaseduration of disease

EndocrineEndocrineDiabetes MellitusDiabetes Mellitus

Nonproliferative diabetic retinopathy (NPDR)Nonproliferative diabetic retinopathy (NPDR)

– – Capillaries develop leaks and occludeCapillaries develop leaks and occlude

– – See microaneurysms, dot/blot hemorrhages, See microaneurysms, dot/blot hemorrhages, exudates, and macular edemaexudates, and macular edema

– – Vision loss at this stage is from macular edemaVision loss at this stage is from macular edema

Diabetes MellitusDiabetes Mellitus

NPDRNPDR

Macular EdemaMacular Edema

EndocrineEndocrineDiabetes MellitusDiabetes Mellitus

NPDRNPDR

– – As it becomes more severeAs it becomes more severe

• • Marked vascular tortuosityMarked vascular tortuosity

• • More hemorrhagesMore hemorrhages

• • Cotton wool spots Cotton wool spots

EndocrineEndocrineDiabetes MellitusDiabetes Mellitus

Proliferative diabetic retinopathy (PDR)Proliferative diabetic retinopathy (PDR)

– – Due to ischemiaDue to ischemia

– – Neovascularization - usually on nerve headNeovascularization - usually on nerve head

– – Vessels bleed into vitreousVessels bleed into vitreous

– – Fibrous tissue develops and causes tractionalFibrous tissue develops and causes tractional

retinal detachmentsretinal detachments

– – PROFOUND visual loss PROFOUND visual loss

PDRPDR

Vitreous HemorrhageVitreous Hemorrhage

Iris NeovascularizationIris Neovascularization

Tractional Retinal DetachmentTractional Retinal Detachment

EndocrineEndocrineDiabetes MellitusDiabetes Mellitus

PDRPDR

– – Needs prompt treatment to prevent Needs prompt treatment to prevent vitreous vitreous hemorrhages and retinal hemorrhages and retinal detachmentsdetachments

– – Treatment causes regression of the Treatment causes regression of the vesselsvessels

– – Treatment reduces visual loss by 50%Treatment reduces visual loss by 50%

EndocrineEndocrineDiabetes MellitusDiabetes Mellitus

PDRPDR– – TreatmentTreatment

• • Scatter 1000-3000 laser burns Scatter 1000-3000 laser burns throughout retinathroughout retina• • Avoid maculaAvoid macula• • Decreases retinal oxygen need which Decreases retinal oxygen need which decreases the impetus for decreases the impetus for

neovascularizationneovascularization• • Treatment needs to be ongoing Treatment needs to be ongoing

Laser effectivenessLaser effectiveness

EndocrineEndocrineDiabetes MellitusDiabetes Mellitus

The only thing that has been shown to delay the The only thing that has been shown to delay the onset of and/or decrease the severity of diabetic onset of and/or decrease the severity of diabetic eye disease is controlling the blood sugar - up to eye disease is controlling the blood sugar - up to 76% reduction!76% reduction!

All diabetics need an eye exam at least once per All diabetics need an eye exam at least once per yearyear

Autoimmune DisordersAutoimmune Disorders

Connective tissue diseases - Connective tissue diseases - SLE, SjogrenSLE, Sjogren’’s Syndrome, s Syndrome,

Ankylosing SpondylitisAnkylosing Spondylitis GravesGraves’’ disease disease Myasthenia gravisMyasthenia gravis Rheumatoid arthritisRheumatoid arthritis Giant cell arteritisGiant cell arteritis

AutoimmuneAutoimmuneConnective Tissue DiseasesConnective Tissue Diseases

Dry eyes are the most common manifestationDry eyes are the most common manifestation Symptoms:Symptoms:

– – BurningBurning

– – PhotophobiaPhotophobia

– – Foreign body sensationForeign body sensation

Autoimmune SLEAutoimmune SLE

Dry eyesDry eyes ScleritisScleritis Peripheral corneal ulcersPeripheral corneal ulcers RetinopathyRetinopathy Optic neuropathyOptic neuropathy

SLE RetinopathySLE Retinopathy

SLE Optic NeuropathySLE Optic Neuropathy

AutoimmuneAutoimmuneGravesGraves’’ disease disease

Improperly called Improperly called ““thyroid eye diseasethyroid eye disease”” Immune system attacks both thyroid glandImmune system attacks both thyroid gland

and extraocular muscles (EOM)and extraocular muscles (EOM) Not related to thyroid hormone levelsNot related to thyroid hormone levels Can progress even when euthyroid Can progress even when euthyroid

AutoimmuneAutoimmuneGravesGraves’’ disease disease

Manifestations:Manifestations:

– – ProptosisProptosis

– – Lid retractionLid retraction

– – Exposure keratopathyExposure keratopathy

– – Strabismus (misaligned eyes)Strabismus (misaligned eyes)

– – Optic neuropathy Optic neuropathy

GravesGraves’’ Ophthalmopathy Ophthalmopathy

GravesGraves’’ Ophthalmopathy Ophthalmopathy

GravesGraves’’ Ophthalmopathy Ophthalmopathy

GravesGraves’’ Ophthalmopathy Ophthalmopathy

Autoimmune Autoimmune GravesGraves’’ disease disease

Treatment - as neededTreatment - as needed

– – Artificial tearsArtificial tears

– – Oral steroidsOral steroids

– – Strabismus surgeryStrabismus surgery

– – Lid surgeryLid surgery

– – Orbital decompression surgeryOrbital decompression surgery

– – Orbital radiation Orbital radiation

Autoimmune Autoimmune Myasthenia gravisMyasthenia gravis

Autoimmune reaction to the acetylcholineAutoimmune reaction to the acetylcholinereceptors in muscle tissuereceptors in muscle tissue

Ocular manifestations:Ocular manifestations:– – PtosisPtosis– – StrabismusStrabismus– – Facial weaknessFacial weakness– – Worse at the end of the day - fatigues Worse at the end of the day - fatigues easily easily

Myasthenia gravisMyasthenia gravis

Autoimmune Autoimmune Myasthenia gravisMyasthenia gravis

Other manifestations:Other manifestations:

– – Proximal limb weaknessProximal limb weakness

– – Difficulty swallowing or breathing (which can Difficulty swallowing or breathing (which can be deadly)be deadly)

– – Sometimes triggered by an underlyingSometimes triggered by an underlying

thymoma - always get chest CT thymoma - always get chest CT

AutoimmuneAutoimmuneMyasthenia gravisMyasthenia gravis

Confirm diagnosis with edrophonium (Tensilon) Confirm diagnosis with edrophonium (Tensilon) test and checking for acetylcholine receptor test and checking for acetylcholine receptor antibodiesantibodies

Treatment:Treatment:– – Oral anticholinesterase (e.g. pyridostigmine)Oral anticholinesterase (e.g. pyridostigmine)– – SteroidsSteroids– – Immunosuppressive therapyImmunosuppressive therapy– – Surgical removal of any thymomaSurgical removal of any thymoma

AutoimmuneAutoimmuneRheumatoid ArthritisRheumatoid Arthritis

Dry eyesDry eyes EpiscleritisEpiscleritis ScleritisScleritis Corneal ulcerationCorneal ulceration UveitisUveitis

Rheumatoid EpiscleritisRheumatoid Episcleritis

Rheumatoid ScleritisRheumatoid Scleritis

Rheumatoid Necrotizing ScleritisRheumatoid Necrotizing Scleritis

Rheumatoid Scleromalacia PerforansRheumatoid Scleromalacia Perforans

Rheumatoid Corneal Ulceration Rheumatoid Corneal Ulceration

Autoimmune Rheumatoid ArthritisAutoimmune Rheumatoid Arthritis

Treatment:Treatment:

– – Artificial tearsArtificial tears

– – Topical steroidsTopical steroids

– – Oral steroidsOral steroids

– – Immunosuppressive therapyImmunosuppressive therapy Treat in conjunction with a rheumatologistTreat in conjunction with a rheumatologist

Autoimmune Juvenile Rheumatoid Autoimmune Juvenile Rheumatoid ArthritisArthritis

Ocular manifestations:Ocular manifestations:

– – Asymptomatic iritisAsymptomatic iritis

– – Secondary cataracts and glaucomaSecondary cataracts and glaucoma PauciarticularPauciarticular Rheumatoid factor -veRheumatoid factor -ve ANA +ANA +

Idiopathic Disorders With Ocular Idiopathic Disorders With Ocular ManifestationsManifestations

SarcoidosisSarcoidosis Multiple SclerosisMultiple Sclerosis HLA B27-associated disorders - ReiterHLA B27-associated disorders - Reiter’’s,s,

ankylosing spondylitis, etcankylosing spondylitis, etc BehcetBehcet’’s diseases disease WegenerWegener’’s granulomatosiss granulomatosis

Idiopathic SarcoidosisIdiopathic Sarcoidosis

Generalized, multisystem inflammatoryGeneralized, multisystem inflammatory

disorderdisorder 25% exhibit ocular manifestations25% exhibit ocular manifestations Much more common in blacks andMuch more common in blacks and

hispanics than whites hispanics than whites

Idiopathic SarcoidosisIdiopathic Sarcoidosis

Ocular manifestations:Ocular manifestations:– – Orbital and eyelid granulomasOrbital and eyelid granulomas– – Lacrimal gland infiltration - causing dry eyeLacrimal gland infiltration - causing dry eye– – Conjunctival nodulesConjunctival nodules– – Uveitis (intraocular inflammation) - mostUveitis (intraocular inflammation) - most

common manifestationcommon manifestation– – Iris nodulesIris nodules– – RetinopathyRetinopathy

Sarcoid UveitisSarcoid Uveitis

Sarcoid RetinopathySarcoid Retinopathy

Idiopathic SarcoidosisIdiopathic Sarcoidosis

Treatment:Treatment:

– – Topical steroidsTopical steroids

– – Systemic steroidsSystemic steroids

– – Sometimes immunosuppressive therapySometimes immunosuppressive therapy

Infectious Diseases With Ocular Infectious Diseases With Ocular ManifestationsManifestations

HIV/AIDSHIV/AIDS Herpes ZosterHerpes Zoster CandidiasisCandidiasis SyphilisSyphilis TBTB Lyme diseaseLyme disease Many others Many others

Infectious HIV/AIDSInfectious HIV/AIDS

Ocular manifestations:Ocular manifestations:

– – Dry eyeDry eye

– – KaposiKaposi’’s sarcoma of eyelidss sarcoma of eyelids

– – HIV retinopathyHIV retinopathy

– – Cytomegalovirus infection (CMV)Cytomegalovirus infection (CMV)

HIV RetinopathyHIV Retinopathy

Infectious HIV/AIDSInfectious HIV/AIDS

CMV retinitisCMV retinitis– – Leading cause of visual loss in AIDSLeading cause of visual loss in AIDS– – 25% of AIDS patients get it25% of AIDS patients get it– – CD4 <50CD4 <50– – Hemorrhagic necrosis of retinaHemorrhagic necrosis of retina– – Indicates poor prognosis for survivalIndicates poor prognosis for survival– – Fortunately, infrequently seen since advent ofFortunately, infrequently seen since advent of

protease inhibitorsprotease inhibitors

HIV/AIDS: CMV RetinitisHIV/AIDS: CMV Retinitis

Infectious HIV/AIDSInfectious HIV/AIDS

Treatment of CMV retinitis:Treatment of CMV retinitis:

– – IV ganciclovirIV ganciclovir

– – IV foscarnetIV foscarnet

– – Intravitreal ganciclovirIntravitreal ganciclovir

– – Ganciclovir sustained-release intravitrealGanciclovir sustained-release intravitreal

implants implants

Infectious Herpes Zoster Infectious Herpes Zoster OphthalmicusOphthalmicus

10-15% of zoster is ocular10-15% of zoster is ocular Follows distribution of V1Follows distribution of V1 Eye is often involvedEye is often involved

- Roughly 5-10%- Roughly 5-10%

- Roughly 40% if tip of nose involved- Roughly 40% if tip of nose involved

Herpes Zoster OphthalmicusHerpes Zoster Ophthalmicus

Prevalence increases with agePrevalence increases with age In patients under 40 - suspect HIVIn patients under 40 - suspect HIV

Herpes Zoster OphthalmicusHerpes Zoster Ophthalmicus

Painful, crusty, vesicular lesions alongPainful, crusty, vesicular lesions along

distribution of nervedistribution of nerve Exam (of eyes):Exam (of eyes):

– – Corneal stromal keratitis (melting)Corneal stromal keratitis (melting)

– – UveitisUveitis

– – RetinitisRetinitis

– – Neuritis Neuritis

Herpes Zoster OphthalmicusHerpes Zoster Ophthalmicus

Herpes Zoster Ophthalmicus Herpes Zoster Ophthalmicus

Herpes Zoster OphthalmicusHerpes Zoster Ophthalmicus

Treatment:Treatment:

– – Oral acyclovir 800mg 5x/day (or IV)Oral acyclovir 800mg 5x/day (or IV)

– – Should be started within 7 days of onsetShould be started within 7 days of onset

– – With eye involvement:With eye involvement:

• • Topical steroids (not trifluridine)Topical steroids (not trifluridine)

• • LubricationLubrication