ocular manifestations of systemic disease - cecentral.com · objectives 1. to describe the ocular...
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Ocular Manifestations
of Systemic Disease
Jacob J. Yunker, MDDiseases and Surgery of the Retina, Macula &
VitreousAssistant Professor, Department of Ophthalmology
University of Kentucky College of Medicine
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Objectives
1. To describe the ocular signs and symptoms associated with selected systemic diseases and their serious ocular sequelae.
2. To review the important features of diabetic retinopathy and the current screening guidelines
3. To be familiar with the important ocular features of hypertension, thyroid disease, sarcoidosis and inflammatory conditions, malignancy and acquired immunodeficiency syndrome.
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Introduction
“The eyes are the window to the soul.”
--English proverb
Numerous systemic diseases have ocular manifestations
Occasionally, the eye findings may be the first indication of underlying systemic disease leading to diagnosis
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Differential Diagnosis
Congenital
Vascular
Traumatic
Neoplastic
Autoimmune
Idiopathic
Infectious
Metabolic/Endocrine
Drugs/Toxins
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Ocular Anatomy
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Anatomy: Periocular soft tissues
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Anatomy: Lacrimal System
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Anatomy: The Globe
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Anatomy: The Retina
The macula = central vision
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Examination
Vision
External
Pupils
Relative Afferent pupillary defect (RAPD)
Motility
Exam of Anterior Segment (Slit Lamp)
Dilated Ophthalmoscopy
Visual Fields
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Hypertension
Systolic > 130, Diastolic > 85 mmHg
65 million Americans
Heart, kidneys, brain, & Eye
May cause damage to retina, choroid, and optic nerve
Acute & Chronic changes
Preeclampsia
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Hypertension
Ocular findings
Arteriolar narrowing & A-V Nicking
Cotton Wool spots
Retinal hemorrhages
Optic nerve swelling (edema)
Retinal ischemia & neovascularization
May be associated with:
Branch retinal artery occlusion (BRAO)
Branch retinal vein occlusion (BRVO)
Central retinal vein occlusion (CRVO)
Retinal artery macroaneurysm (RAMA)
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Hypertensive Retinopathy
Images courtesy of Redatlas.org Cotton wool spots
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Hypertensive Retinopathy
Optic Nerve Edema
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Hypertensive Retinopathy
BRAOCRVO
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Diabetes mellitus
Diabetic retinopathy (DR) is leading cause of blindness in 20-64yo in US
Duration of DM is directly related to prevalence of retinopathy
After 20 yrs: 99% of type 1 and 60% of type 2 will have some degree of DR
Pathologic changes include microvascular damage leading to hypoxia, vascular leakage & edema, ischemia, and neovascularization
Prevention & Importance of systemic blood glucose, pressure, & lipid control: DCCT, UKPDS, & ACCORD trials
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Diabetic Retinopathy
Classification:
Non-Proliferative diabetic retinopathy (NPDR)
Proliferative Diabetic Retinopathy (PDR)
Complications Resulting in Visual Loss
Macular edema (capillary leakage)
Macular ischemia (capillary occlusion)
Sequelae from ischemia-induced neovascularization
NPDR & PDR
PDR
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Diabetic Retinopathy:NPDR
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Diabetic Retinopathy:Macular Edema
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Diabetic Retinopathy:PDR
NVD NVE
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Diabetic Retinopathy:PDR
NVE
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Diabetic Retinopathy:PDR
Vitreous Hemorrhage
Tractional Retinal Detachment
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Diabetic Retinopathy:Treatment
Laser
Pharmacologic (anti-VEGF meds)
Surgery
Laser Pan Retinal Photocoagulation
Vitrectomy Surgery
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Diabetic Retinopathy:Vitrectomy Surgery
Scar tissue, membranes,
& blood on surface of
retina
Neovascularization (PDR)
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Diabetic Retinopathy:Screening & Prevention
Screening eye examination for all patients:
At time of diagnosis for type 2
Within 5 yrs of diagnosis for type 1
Pregnancy
Follow-up exams will be dependent on initial eye findings
Blood glucose, Blood pressure, and Lipid optimization
Stop smoking
Exercise
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Carotid Stenosis
Amaurosis fugax
Hollenhorst plaque
Retinal artery occlusion
Ocular ischemic syndrome (& neovascular glaucoma)
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Hollenhorst plaque
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Carotid Stenosis:Branch Retinal Artery Occlusion
Embolus
Retinal whitening due to edema
Embolus
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Ocular Ischemic Syndrome
Pain
Red Eye
Loss of vision
Elevated Intraocular Pressure
Patients at risk for ischemic
CV disease, stroke, &
peripheral Vascular disease
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Sickle Cell Disease
Autosomal Recessive
Genotype
8% Sickle cell trait (HbAS)
0.4% Sickle cell disease (HbSS)
0.2% SC disease (HbSC)
Sickle Thalassemia
Systemic Complications
Crises (painful episodes)
Acute chest syndrome
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Sickle Cell Disease:Ocular Complications
Frequency
HbSC (most common) – 33%
Sickle Thalassemia – 14%
HbSS – less frequently but have more severe systemic complications – 3%
Pathogenesis:
Peripheral retinal nonperfusion – ISCHEMIA =
Neovascularization Hemorrhage & Tractional
Retinal Detachment
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Sickle Cell Disease
Capillary nonperfusion in peripheral retina
Neovascularization
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Sickle Cell Disease
Neovascularizaiton
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Sickle Cell Disease:Special Situations
High Risk for elevated intraocular pressure due to sickling of RBCs
Require aggressive medical & sometimes surgical Rx to prevent optic nerve damage
Know Sickle Cell Status for patients with Traumatic Hyphema
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Giant Cell Arteritis
AKA: Temporal Arteritis
Systemic granulomatous vasculitis affecting medium- and large-sized vessels
Age > 50 yo
Symptoms Headache
Scalp tenderness
Jaw claudication
Polymyalgia rheumatica
Acute vision loss
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Giant Cell Arteritis
Diagnosis:
Clinical history
Stat ESR &/or CRP
Temporal artery biopsy
Treatment:
High-dose systemic steroids (do not defer until after biopsy)
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Giant Cell Arteritis
Dilated superficial temporal artery
Scalp Necrosis
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Giant Cell Arteritis
Central Retinal Artery Occlusion
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Temporal Artery Biopsy
Superficial Temporal artery
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Thyroid Eye disease
May occur with hyper-, hypo-, or euthyroid states
Hyperthyroidism: goiter, tremor, pretibial myxedema, atrial fibrillation, etc
Ocular Findings:
Proptosis (exophthalmos)
Lid Retraction -- Thyroid stare
Corneal exposure (dry eye, corneal ulcer)
Diplopia (due to eye muscle restriction)
Optic Nerve compression (optic neuropathy) – 5%
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Thyroid Eye Disease:Pathophysiology
Autoimmune process with cross-reaction against orbital and periorbital soft tissues
Enlargement of ocular rectus muscles
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Exophthalmos:“Thyroid Stare”
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Thyroid Eye disease
Chemosis & Dry Eye
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Thyroid Eye disease
CT scan orbits or Orbital Ultrasound
Look for enlargement of eye muscles
Restrictive myopathy = Double Vision
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Thyroid Eye disease:Orbital Decompression
(For TED-related Optic Neuropathy)
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Thyroid Eye disease:Management
Treatment Considerations:
Artificial tears & lubrication
Systemic steroids & external beam radiation (if vision threatening)
Surgery:
Orbital decompression
Eye muscle surgery
Eyelid Surgery
Stop Smoking
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HIV & AIDS
Eyelids: Kaposi sarcoma, molluscum contagiosum, Herpes zoster ophthalmicus
Orbit: Cellulitis, B-cell lymphoma
Cornea: Keratitis (microsporidium, HSV, HZV)
Keratoconjunctivitis sicca (severe dry eye)
Anterior uveitis
HIV retinopathy
Retinitis (CMV, VZV, toxoplasmosis)
Choroiditis (pneumocystis, toxoplamosis)
B-cell intraocular lymphoma
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Kaposi Sarcoma
Purplish red to bright red highly vascular lesions with surrounding telangiectatic vessels
Associated with Human Herpes Virus-8 (HHV-8)
20-24% of AIDS-related Kaposi sarcoma will involve eye
Eyelid & Conjunctiva
Mostly local mass effects – pain, poor eyelid closure, etc
Treatment: chemotherapy, surgical (if large to debulk)
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Kaposi Sarcoma
Photos courtesy of Gary N Holland, MD, University of California, Los Angeles, Department of Ophthalmology, Jules Stein Eye Institute
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HIV/AIDS:Molluscum contagiosum
Typically multiple lesions in HIV or AIDS
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HIV/AIDS:Herpes Zoster
Hutchinson’s sign
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AIDS:CMV Retinitis
Most common intraocular infection with AIDS
Much reduced incidence since HAART (50% to 10% of pts)
CD4 count typically < 50 cells/mm3
Retinal necrosis, exudation, & hemorrhage
Treatment:
IV ganciclovir/foscarnet
Intravitreal ganciclovir/foscarnet; Ganciclovir intravitreal implant
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AIDS:CMV Retinitis
CD4 < 50
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HIV/AIDS:Toxoplasmosis Retinitis
CD4<100
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Syphilis
= Tertiary Syphilis
Need LP
Rx with IV Penicilin G
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Tuberculosis
Granulomas = Choroidal Tubercules
Uveitis
Choroidal granulomas
Periphlebitis
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Septicemia & Endogenous Endophthalmitis
Hematologic seeding of the eye with infection
Gram-positive, gram-negative, fungal etiologies
Candida is most common causative organism
Immunocompromised or immunosuppressed patients
Chronic diseases (DM, Renal failure) & indwelling lines or invasive procedures, & intravenous hyperalimentation
IV Drug Use
Bilateral in 25%
Clincial scenarios: Endocarditis, pneumonia, indwelling catheter (fungal), following surgery (cardiac, pulmonary, urologic)
Treatment: IV Anti-bacterial/fungal Adjunctive: +/- intravitreal
antibiotics, Vitrectomy surgery
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Septicemia & Endogenous Endophthalmitis
Hypopyon
Pneumonia
Bilateral Endophthalmitis
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Septicemia & Endogenous Endophthalmitis
Fungal Endophthalmitis
Vitreous Haze & Debris
(Obscures view of retina)
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Ankylosing spondylitis
Spondylarthropathy of the axial skeleton
Typically affects males (4:1)
90% are HLA-B27 (+)
Presents in early adulthood (15-35 yo) with pain & stiffness in lower back
Limitation of spinal flexion
Juxta-articular osteoporosis & fusion of sacro-iliac joints
“Bamboo spine”
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Ankylosing spondylitis
Ophthalmic features:
Anterior uveitis in 30-40%
Symptoms
Photophobia
Redness
Decreased vision
Treatment:
Topical corticosteroids
Cylcoplegia
Fusion of sacro-iliac joints
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Ankylosing spondylitis
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Behcet’s Disease
Vasculitis leading to chronic inflammation & ulceration
Oral aphthaous ulcers
Genital ulcers
Skin lesions (e.g. erythema nodosum)
Eye inflammation (iritis, retinal vasculitis)
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Behcet’s Disease
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Inflammatory Bowel Disease (IBD):Crohn’s Disease & Ulcerative Colitis
Ulcerative colitis: relapsing, non-transmural, restricted to colon
Crohn’s disease: relapsing, transmural, affects entire GI tract
Ocular complications in 10%
Uveitis
Episcleritis
Scleritis
Women at higher risk
Associated with HLA-B27
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Sarcoidosis
Focal noncaseating granulomas
AA females, 20-40 yo
Elevated Calcium, ACE, abnormal CXR
Lacrimal gland granulomas
Anterior uveitis
Retinal inflammation
Ocular complications: glaucoma, cataract
Hilar Lymphadenopathy
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Sarcoidosis
Lacrimal Gland Granuloma
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Sarcoidosis
“Mutton fat” KP
(Keratitic Precipitates)
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Sarcoidosis
Posterior Synechiae of the iris
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Sarcoidosis
Granuloma of optic nervePerivascular Inflammation
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Rheumatoid Arthritis
25% may have ocular findings
Dry eyes (15-25%)
Episcleritis
Scleritis
Corneal ulcers
uveitis
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Rheumatoid Arthritis
Peripheral Ulcerative Keratitis
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Rheumatoid Arthritis:Scleritis
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Multiple Sclerosis:Optic Neuritis
50% of patients with MS will develop Optic Neuritis
20-30% of time will be presenting sign for MS
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Metastatic Disease
Most common intraocular malignancy in adults
May be asymptomatic
May produce decreased or distorted vision
Most common primary: Lung, Breast
10% have unknown primary
No prior history of Cancer in 25%
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Metastatic Lung Cancer
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Metastatic Breast Cancer
Elevated Amelanotic Mass in Macula S/p ChemoRx
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Myasthenia gravis
Autoimmune neuromuscular disorder leading to fluctuating muscle weakness & fatiguability
Circulating antibodies block Ach receptors at post-synaptic NM junction, inhibiting stimulative effect of neurotransmitter Ach
Ptosis, double vision, problems chewing, talking, and swallowing
Diagnosis: Tensilon test, single-muscle fiber EMG
Treatment: Acetylcholinesterase inhibitor (Mestinon)
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Myasthenia gravis
Ptosis
Fatiguability
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Neurofibromatosis-1
Autosomal Dominant
Chromosome 17
Diagnostic Criteria
Café au lait spots
Intertrigenous freckle
Neurofibroma
Optic nerve glioma
Lisch nodules
Osseous leasions
Family history in 1st degree relative
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Neurofibromatosis-1
Seen in 90% of cases
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Neurofibromatosis-1
S-Shaped Eyelid Neurofibroma
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Neurofibromatosis-1
Optic Nerve Glioma
20% with NF-1
will have Optic
Nerve glioma
50% of pts with
optic nerve
glioma have NF-
1
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Sturge-Weber Syndrome
Sporadically inherited phakomatoses
Glaucoma
Dilated & tortuous episcleral vessels
Seizures
CNS angiomas (Leptomeningeal hemangioma)
calcification
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Sturge-Weber Syndrome
Nevus flammeus (Port Wine Stain)
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Sturge-Weber Syndrome:Choroidal Hemangioma
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Systemic Medications
Benign ocular conditions
Amiodarone – whorl keratopathy
Toxic Retinopathies
Thioridazine, chloroquine, hydroxychloroquine, tamoxifen
Toxic Optic Neuropathies
Ethambutol, isoniazid
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Hydroxychloroquine: Ophthalmic Screening
Used for rheumatoid arthritis, SLE, etc
Ocular toxicity rare with usual dose 200 mg bid (5-7mg/kg/day)
Toxicity related to cumulative dose (>460 g) & duration of use
Ocular findings: bulls-eye retinopathy
Recommended screening:
Baseline exam & Central VF testing
Annual examination & repeat central VF for pts using
medicine > 5 yrs
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Plaquenil toxicity:Bull’s Eye Maculopathy
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Thank You for your Attention
???Any Questions???