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Systemic Disease SECO

VIEWS: 53 PAGES: 45

									                                                     SECO 2011
DISCLOSURE STATEMENT                                                 March 2-6, 2011

  Dr. Pizzimenti is CEO of Optometryboardcertified.com                                        • The Eye is an extension of the brain
  Dr. Pizzimenti has received honoaraia from Alcon, Reichert,
  Zeavision, and Carl Zeiss Meditec                                                                                                                                  retina”
                                                                                              • The anatomy of the eye is structured to serve the functions of the “ retina”
  Dr. Pelino has received honoraria from Carl Zeiss Meditec
                                                                                              • The primary reason for dilation is to detect systemic disease
              Essentials in Systemic Disease
                           Carlo J. Pelino, OD
                        Joseph J. Pizzimenti, OD
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Welcome to SECO 2009                     At the conclusion of this course, please properly
                                         dispose of your trash as you leave this room




                                                                                             Inner and Outer blood retinal barrier      RPE and Choroidal pigmentation




                                                                                                                                                                               1
                                                            Choroid 80%

                                                            Sympathetic
                                                            Control



                                                             Retina 5 %

                                                             Autoregulate




                    Iris / Ciliary Body 15%




Classification of Blood Pressure (Adults 18 and older)
                                                                            Clinical Ophthalmoscopic findings
Category         Systolic Blood Pressure Diastolic Blood Pressure
                                                                            Grading of Hypertensive Retinopathy
• Nomal              < 120 mm Hg                    < 80 mm Hg

• Prehypertension         120-139 mm Hg             80-89 mm Hg
                                                                            Grade 1          Retinal vessels narrowed            > 90 and < 110 Diastolic BP
• Hypertension      140-159 mm Hg             90-99 mm Hg
                                                                            Grade 2          Nicking of retinal vessels          > 90 and <110 Diastolic BP
  (Stage 1)
                                                                            Grade 3               Hemes,
                                                                                             CWS, Hemes, Lipid exudates          > 110 – 115 Diastolic BP
• Hypertension      >160 mm Hg                100 mm Hg
  (Stage 2)                                                                 Grade 4          Grade 3 + Optic disc swelling       > 130 Diastolic BP

Source: U.S. Department of Health Services; NIH no. 03-5231 May 2003           • Grades 3 and 4 = increase risk of cerebral, heart and kidney problems




                                                                                                                                                               2
54 year old

+ Diabetes

+ HTN

+ Cholesterol




                3
4
5
Grade 4 Hypertensive Retinopathy




                                   6
Malignant Hypertension   Malignant Hypertension




Malignant Hypertension




                                                  7
          Hypertension:
Malignant Hypertension:


    • Defined as Blood Pressure > 210 / 130


       Presentation:
Ocular Presentation: May present with the following

    • Disc Edema
    • Arterio-venous crossing changes
    • Nerve fiber layer infarcts (cotton wool spots)
    • Macular edema
    • Hard exudates
    • Flame shaped hemorrhages
    • Choroidal ischemia (usually seen in young patients)




                                                            8
                                                                                 • Keith et al. found that ~ 80 % of patients with Grade 4 Hypertensive
           Hypertension:
 Malignant Hypertension: Systemic Presentation                                    Retinopathy ( malignant hypertension ) died within 1 year

      • Patient may be asymptomatic                                              • Also, over a 3 year period, there was a 95% mortality for those with
      • Patient may have encephalopathy, headaches, vomiting or coma               malignant hypertension

                                                                                 • HLA B15 has been associated with malignant arterial hypertension.
Treatment of Malignant Hypertension:

     • Blood pressure measurement
     • Immediate referral to emergency room or primary care doctor for
          slow lowering of the blood pressure !!!!!

     • Visual field testing
     • MRI – to rule out space occupying lesion
     • MRV- to rule out “ venous sinus thrombosis ”
     • Lumbar puncture if necessary




Hypertension:
Hypertension      ~ 70 million Americans
                                                                                               Hypertension:
                                                                                     Secondary Hypertension:
    • Malignant
    • Essential                                                                      Drugs / Toxins
                      Pheochromocytoma,              stenosis,
    • Secondary HTN ( Pheochromocytoma, renal artery stenosis, etc. )
                                                                                     Renal Disease             Glomerulonephritis,
                                                                                                               Glomerulonephritis, Diabetes, Chronic nephritis
          Hypertension:
Essential Hypertension: defined as a blood pressure > 140 / 90
                                                                                     Vascular                  Coarctation of the aorta
                                      age…
•During young adulthood /early middle age…HTN is more common in males
                                                                                     Neurologic                Increased Intracranial Pressure, G-B syndrome
         patients…
•Elderly patients…HTN is more common in women
                                                                                     Endocrine                 Pheochromocytoma,
                                                                                                               Pheochromocytoma, Hypo-Hyperthyroidism
•A family history of HTN usually exists in essential HTN
•Essential HTN is usually controlled with one or two medications
                                                                                     Pregnancy
•Blood pressure does not progress to higher levels over a short period of time
                                                                                     Stress                    Postoperative, Burns, Alchohol withdrawal
                                                                 Panel”
•Work Up = BUN/Cr, Lipid Profile, Glucose, CBC, EKG, “ Metabolic Panel”
                                                                                 Zoorob RJ, et al. Hypertension. Prim Care. 2000 Sep;27(3):589-614




                                                                                                                                                                 9
Always question the malignant hypertensive patient
    Hypertensive encephalopathy
       • Syncope                                                                       2 Months
       • Seizures
       • Focal weakness
       • Paresthesias
       • Speech problems

   Hypertensive Cardiac involvement
      • Chest pain
      • Palpatations
      • Cough
      • Dyspnea

   Hypertensive renal problems                                                         BP 125 / 82
      • Change in renal volume                                                         RAS
        Hematuria,
      • Hematuria, abdominal pain




                                                        • Hypertensive Choroidopathy
                        Epiretinal Membrane formation




                                                                                                     10
Essential Hypertension – Long standing




                    Arteriosclerosis Grade 2-3




                Retinal Arterial Macroaneurysm   Retinal Arterial Macroaneurysm




                                                                                  11
   Central Retinal Vein Occlusion   Branch Retinal Vein Occlusion
                                                                                      Ischemic”
                                                                               “Non - Ischemic” Central Retinal Vein Occlusion




Valsalva Retinopathy
                                                                    Valsalva Retinopathy

                                                                       • Rupture of the superficial retinal capillaries

                                                                       • Occurs when there is a rise in the intrathoracic or intra-abdominal
                                                                         pressure

                                                                       • There is then a rise in the intraocular venous pressure

                                                                       • Increased pressure is generated by forceful exhalation against a closed
                                                                         glottis

                                                                       • Etiology may be from strenuous coughing, sneezing, vomiting,
                                                                         straining or lifting




                                                                                                                                                   12
                                                                                     1 Week
  Day #1




BP 162 / 98
                                                                                    BP 135 / 90
RAS
                                                                                    RAS
35 yo AA male




                  Valsalva Retinopathy

    4 Weeks        Treatment:
                   Treatment:
                       • Usually observation – Most hemorrhages clear spontaneously and have
                            an excellent prognosis
                       • Vitrectomy
                       • Nd:YAG laser – disruption to the internal limiting membrane used to
                            disperse the dense hemorrhage into the inferior vitreous

                             Note:
                   Important Note:
                      • Toxic damage to the retina if contact with hemoglobin and iron for a
      BP 140/90         long period of time
      RAS
                            Evaluation:
                   Systemic Evaluation:
                                                                           pressure, coagulopathy,
                       • A work up should include testing for high blood pressure, coagulopathy,
                         and blood dyscrasias especially if positive for a family history




                                                                                                     13
                                     State:
                      Hypercoaguable State:

                      • It is a risk factor for artery and venous occlusions
                      • Has and association with coronary artery disease
                      • Has and association with cerebral vascular accidents (CVA)
                      • Hypercoaguable state is associated with peripheral vascular disease


                              State:
                      Primary State:                                        State:
                                                                  Secondary State:
                      • Protein C deficiency                        • Pregnancy
                      • Protein S deficiency                        • Malignancy
                      • Antithrombin III                            • Congestive Heart Failure
                      • Factor V Leiden                             • Immobility
                      • Hyperhomocysteinemia
                      • Prothrombin 20210 mutation
                                                                            Anti-cardiolipin
                      • Antiphospholipid syndrome (Lupus anticoagulant / Anti-cardiolipin antibody)
                      • CBC c differential, Platelet count and PT / PTT




Coagulation Pathway

                                      State:
                       Hypercoaguable State: Important Note

                           • Factor V Leiden is the most common hereditary blood coagulation
                               disorder in the United States ~10%

                           • Prothrombin 20210 mutation is the second most common inherited
                               clotting abnormality in the United States


                       Ophthalmic Presentations:

                           • Central Retinal Artery Occlusion
                           • Branch Retinal Artery Occlusion
                           • Central Retinal Vein Occlusion




                                                                                                      14
 BRAO”
“BRAO” in a 42 yo AA female
  Birth-control medication




                              15
               State:
Hypercoaguable State: Treatment

      •Monitor patient closely with Primary Care Physician

       Coumadin,
      •Coumadin, Heparin, Aspirin therapy

      •Treat ocular conditions accordingly




                                                             Dot and Blot hemes in mid-peripheral retina




                                                                                                           16
                                            Syndrome:
Hypoperfusion Retinopathy / Ocular Ischemic Syndrome:


   • Usually unilateral but may be bilateral in 20% of cases

   • Males > Females by a 2 to 1 ratio

   • Dot and blot hemes / microanuerysms found only in the mid-
   peripheralretina = Hypoperfusion Retinopathy

   • When the above is associated with neovascularization of the Disc,
     Retina, Iris or Angle = Ocular Ischemic Syndrome




                                                                         17
Pathogenesis:
Pathogenesis: Ocular Ischemic Syndrome:
                                                                   Symptoms:
                                                                   Symptoms: Ocular Ischemic Syndrome
• Atheromatous ulceration and stenosis at the bifurcation of the
  common carotid artery ( 90% occlusion has to be present )
                                                                                                                              Angina”
                                                                      • Ocular and periorbital pain in 40% of cases = “Ocular Angina”

                                                                      • Prolonged recovery of vision following exposure to bright light-
                                                                                                     Amaurosis”
                                                                          known as “ Light Induced Amaurosis”

                                                                      • Amaurosis Fugax ( Transient Monocular Blindness ) in 5% of cases

                                                                      • Transient Ischemic Attacks (TIA)

                                                                      • Vision Loss ( 90% ) – Short Posterior Ciliary Arterial hypoperfusion




               Carotid Doppler                                                          Carotid Bruit




                                                                                                                                               18
Symptoms: Ocular Ischemic Syndrome
Symptoms:                                                                           Signs:
                                                                             Ocular Signs: Ocular Ischemic Syndrome:

                                                                             • Dilated but not tortuous retinal veins
                                                                             • Retinal Hemorrhages in mid-peripheral retina (80%) of patients
                                                       Angina”
 Ocular and periorbital pain in 40% of cases = “Ocular Angina”               • Cotton Wool Spots (5%)
                                                                             • Neovascularization of the Disc (35%)
                                                                             • Neovascularization of the Retina (8%)
         • Ischemia to the ophthalmic division of Cranial Nerve 5            • Rubeosis iridis (65%)
                                                                             • Uveitis – mild anterior (20%)
         • Anterior segment inflammation                                     • Emboli (retinal)
                                                                             • Lower IOP - initially
         • Elevated intraocular pressure ( IOP ) from neovascular glaucoma




                                                                                                                                                19
Ocular Ischemic Syndrome




                 Cholesterol Plaques
                                       55 yo AA male “BRAO” OD




                    55 yo AA male OS




                                                                 20
Cerebral Blood Supply   Motor and Sensory Areas




                                                  21
                                                                               Ocular Ischemic Syndrome

                                                                                    Up:
                                                                               Work Up:

                                                                                   • Carotid artery evaluation (Carotid – Duplex Scanning) – ICA, ECA,
                                                                                   CC

                                                                                   • Color Trans-cranial doppler (TCD) – ocular arteries

                                                                                   • Possible MRA (Magnetic Resonance Angiography)

                                                                                   • Computed Tomography (CT) angiography

                                                                                   • Cardiology work up (Echocardiogram) – Transesophogeal /
                                                                                   Transthoracic

                         Occipital Lobe Infarct                                    • HTN, DM, Lipid Panel, ESR, C-reactive protein




Ocular Ischemic Syndrome                                                       Diabetes

    Treatment:
    Treatment:
                                                                               Characteristics
                                                (Endarterectomy)
        • Consider carotid surgery if warranted (Endarterectomy)
                    European Carotid Surgery Trial (ECST)
                    North American Symptomatic Carotid End. Trial
                    ( NASCET)                                                  • ~ 24 million American have Diabetes


        • Therapeutic approach – Aspirin ( 325 mg QD or BID ) , Plavix         • Most common retinal vasculature disease
        • Control modifiable vascular risk factors ( HTN, DM, dyslipidemia )
        • Stop smoking                                                         • Diabetes = leading cause of blindness in Americans between 20-74
        • Panretinal photocoagulation (PRP) if neovascularization                years old

    Important Note:
              Note:                                                            • African Americans, Hispanics and Native Americans are high risk groups
           Leading cause of death = Ischemic heart disease
           Second leading cause of death = Stroke                              • Non white females are at greatest risk for blindness




                                                                                                                                                          22
                                                                           Diabetes
The Prevalence of Diabetic Retinopathy
                                                                                Classification of Diabetic Retinopathy

                                                                                 Non-Proliferative”
                                                                                “Non-Proliferative”                       Proliferative”
                                                                                                                         “Proliferative”
• Hispanic population tends to have the highest prevalence rates of DR
                                                                                • Mild                                   Early
• African Americans tend to have highest rates of vision threatening DR
                                                                                • Moderate                               High risk
                                                                                • Severe (4-2-1 rule)
• No prominent difference between genders were seen in the prevalence of
                                                                                • Very Severe
  diabetic retinopathy

• The prevalence of diabetic retinopathy is in older age groups                 Other Ocular Complications:
                                                                                   • Clinically Significant Macular Edema
                                                                                   • Diabetic Papillopathy
                                                                                   • Cranial Nerve Palsy 3,4,6
                                                                                   • Cataract formation




                                                                            Diabetes




                                                                                        Severe”
                                                                                       “Severe” Non-Proliferative Diabetic Retinopathy




                                                                                                                                           23
                                                  Diabetes




                                                                   Proliferative”
                                                                  “Proliferative” Diabetic Retinopathy




                                                   Diabetes
Diabetes




                                                  Dehemoglobinized Vitreal Heme          Proliferative”
                                                                                        “Proliferative” Diabetic Retinopathy
            Proliferative”
           “Proliferative” Diabetic Retinopathy




                                                                                                                               24
Diabetes                                                    Diabetes




                                                                                             Proliferative”
                                                                    Fibrous Proliferation – “Proliferative” Diabetic Retinopathy
                  Proliferative”
                 “Proliferative” Diabetic Retinopathy




 Diabetes                                                                            4-2-1 Rule
                                                               Severe NPDR
                                                               At least one of :

                                                                   intraretinal hemorrhages in four quadrants

                                                                   venous beading in two quadrants

                                                                   intraretinal microvascular abnormalities in 1 quadrant

                                                               Standard photographs available at:
                                                                eyephoto.ophth.wisc.edu/ResearchAreas/Diabetes/DiabS
                                                                tds.htm

             CSME”
            “CSME” – Clinically Significant Macular Edema




                                                                                                                                   25
    Intraretinal hemorrhages in four       Venous Beading and Intraretinal
                quadrants                  Microvascular Anomalies (IRMA)




                                       Venous
                                       beading                 IRMA




DIABETIC MACULAR EDEMA

                                                         CSME Defined
                                          CSME, as defined by the ETDRS, exists
                                           with any of the following findings:
                                              Retinal thickening within 500 mm of the
                                               center of the fovea
                                              Hard exudates within 500 mm of the center of
                                               the fovea with adjacent retinal thickening
                                              At least 1 disc area of retinal thickening, any
                                               part of which is within 1 disc diameter of the
                                               center of the fovea




                                                                                                 26
           Diabetic Macular Edema                                                  Management Guidelines: DME
                                                                                  More recently, laser out-performed
                                                                                                Kenalog.
                                                                                   intravitreal Kenalog.
                                                                                  Laser + Lucentis out-performed laser
                                                                                   alone.


                                                                                                                          +




                                                                              Diabetes
Diabetes
                                                                               #2 Anemia
    Some important systemic effects of diabetes that affect retinopathy and        • Gets more severe as renal disease worsens
                                Attack”
    increase the risk of “Heart Attack”:                                                                    Erythropoietin”
                                                                                   • Kidney production of “Erythropoietin” decrease, which means that less
                                                                                     reaches the bone marrow and less red blood cells are made
    #1 Proteinuria                                                                 • Measured by Hematocrit (HCT) and hemoglobin levels in a CBC
            • First sign of renal disease                                              If hemoglobin levels are less than 11g/dl = anemia
                                increases…
            • As nephropathy increases…the glomerular filtration rate falls
            • American Diabetes Association (ADA) recommends
                      yearly urinalysis                                              Anemia may actually be making the retinopathy worse!!!

            • Random Spot Urine or 24 hour collection
                   Normal <30
                                                                                                           Procrit”                      dialysis…
                                                                                   Treat the patient with “Procrit” if patient is not on dialysis…also give iron
                   Microalbuminuria 30 mg – 299 mg
                   Albuminuria > 300 mg
                                                                                     Kidney           Erythropoietin             Bone Marrow              RBC’
                                                                                                                                                          RBC’s




                                                                                                                                                                   27
Diabetes                                                                                                                  WBC’
                                                                                                                          WBC’s adhere to
                                                                                                                          and accumulate
                                                                                                                          within the retinal
     #3 Hyperlipidemia
                                                                                                                          vasculature
            • Cholesterol and triglyceride healthy levels should be < 200 mg/dl
                                                                                                                          ICAM
            • PCP should consider Lipitor if cholesterol high
                                                                                                                          Further
     #4 Hypertension                                                                                                      breakdown
            • Target blood pressure for diabetics with nephropathy is 130 /80                                             of the blood-
            • ACE inhibitors should be given if blood pressure is high                                                    retinal barrier
                                                              anti-proteinuric
            • ACE inhibitors are both renal-protective and anti-proteinuric
            • Hypertension in diabetics is labile and only effectively
              measured at home                                                                                            Release of VEGF
                                                                                                                          Vascular
     #5 Hyperglycemia                                                                                                     Endothelial
            • Induces vasoconstriction = kidney ( glomerular ) damage                                                     Growth
            • The Hemoglobin A1C should be as close to 7 % as possible                                                    Factor




#6. Obstructive Sleep Apnea                                                       Continuous Positive Airway Pressure

• Progressive relaxation of upper airway musculature in deeper stages of sleep
• Nocturnal Hypertension




                                   REM
                                   Sleep

                                                                                      Nasal Mask:
                                                                                                    • Intimidating
                                                                                                    • Cumbersome
                                                                                                    • ? Poor Compliance




                                                                                                                                               28
                                                                                       DM + Smoking = Blindness



    • Elevated morning BP by 20 -30 mmHg
    • Elevated, resistant BP throughout the day
    • Relationship: Congestive Heart Failure, Nocturnal MI / CVA, Nephropathy




#7. Smoking                                                                     Smoking

        • Severely worsens microangiopathy                                       • Smoking is a risk factor for proteinuria and increased blood pressure

                                                                                 • Nicotine increases blood platelet viscosity which can increase retinopathy

                                                                                 • Smoking also causes arterial wall damage and constriction




                                                                                                                                                                29
 Moderate”
“Moderate” Non-Proliferative Diabetic Retinopathy




                                                    30
31
32
     ACCORD study                                                                 Diabetes
     • Patients with tightly controlled blood glucose (HbA1C < 6%) had a 22%
       higher risk of death vs. those patients with less HbA1C control (7-8%)          Home:
                                                                                  Take Home:

     • The study was halted
                                                                                     • Diabetic Retinopathy is exacerbated by many concomitant conditions
     ADVANCE study

     • Had different results. Risk reduction by 10% in micro and macrovascular       • Control of the systemic aspects of the disease improves the systemic
       events.                                                                         and ocular health

     DPP (Diabetic Prevention Program) - Prediabetics
                                                                                     • Understand how Diabetic Retinopathy relates to the overall systemic
                                                  (metformin)
     • Lifestyle intervention group vs. med group (metformin) vs. placebo              health

     • Lifestyle group showed the most decrease in diabetes risk




Cholesterol /
                                                                   Cholesterol                                    • Arcus Senilis
Triglycerides
< 200 mg/dl                                                        990 mg/dl                                      • Arcus Juvenilis

                                                                                                                  • 50% have high cholesterol levels
Triglycerides                                                      Triglyceride
> 2,500 mg/dl                                                      7,200 mg/dl
                                                                                                                  • In younger people may lead to MI or
                                                                                                                   cardiovascular disease

                                                                                                                  • Arcus = “Bow like

                                                                                                                  • Hyperlipoproteinemia in younger pts.



                                        Retinalis”
                               “Lipemia Retinalis”




                                                                                                                                                              33
The Association of Corneal Arcus with Coronary Heart Disease and
Cardiovascular Disease Mortality in the Lipid Research Clinics                Hyperlipidemia
Mortality Follow-up Study         ( Am J Public Health 1990; 80:1200-1204 )

                                                                                     • Type 2 diabetics have elevated triglyceride levels
 Prospective study of White men ( n = 3,930 )
 and women non-hormone users ( n = 2139 )                                            • Type 2 diabetics have decreased high-density lipoprotein (HDL) levels
 Followed for an average of 8.4 years
                                                                                     • Increases cardiovascular mortality risk = Coronary Artery Disease

  Results:
  Results:
                                                                              Management:
 • Corneal Arcus was strongly associated with CHD and CVD mortality
  only in hyperlipidemic men ages 30-49 years.                                       • Nutrition Therapy = lifestyle change

 • Among 30-49 year old males, corneal arcus was a prognostic factor                 • Exercise = lifestyle change
   for CHD independent of hyperlipidemia
                                                                                     • Drug Therapy                      (aka) HMG-CoA
                                                                                                                 Statins (aka) HMG-CoA reductase
                                                                                                                 inhibitors




Aspirin Use in Diabetes


• Aspirin use in diabetic patients is not associated with an
  increased risk of hemorrhage or progression of retinopathy
  or macular edema !!!

• Aspirin use may actually slow the progression of diabetic retinopathy ???

• Aspirin Therapy ( 81-325 mg/day): ADA recommendations

    • Family History of coronary heart disease
    • Cigarette smoking
    • Hypertension
    • Obesity
    • Albuminuria
    • Elevated lipid levels
    • Age > 30 years




                                                                                                                                                               34
                       29 year old AA female. Dry eye complaints




                             HIV Retinopathy – CD4 count 110
Cotton Wool Spots OU              Pt. not on HAART




                                                                   35
                                                                   • CD4 Count – Measure of T-cell count ( 600 – 1500 cell / m3 )

                    45 year old male. Diabetes history             • CD4 / CD 8 Ratio ( Normal is 2.0 )

                                                                   • Viral Load - # of HIV 1-RNA molecules / ml blood ( 10,000 low / 100,000 high )




                                                                                               Cytomegalo-virus (CMV)




CD 4 Count and Ocular Management of the HIV Patient               Acquired Immune Deficiency Syndrome (AIDS)


                                                                   Course of the Disease

   CD4 Count                           Frequency of Examination
                                                                       • Initial Stage – Influenza like illness ~ 4-12 weeks after becoming infected
   > 250 cell / mm3                            1 year

   150 cell / mm3                              6 months                • Chronic Stage – Latent period ~ 10 years with minor immune dysfunction

   50 -150 cell / mm3                          3 months
                                                                       • Final (Crisis) Stage – Weight loss, fever, skin rashes, opportunistic infections
   < 50 cell / mm3                             1 month                                          and neoplasms




                                                                                                                                                            36
                                                  disease:
Five drugs currently available to people with CMV disease:      www.aidsmeds.com



Foscarnet (Foscavir®)               Through an IV line


Ganciclovir (Cytovene®)             Through an IV line followed by capsules


Cidofovir (Vistide®)                Through an IV line


Valganciclovir (Valcyte®)           Tablets that must be swallowed.


Ganciclovir implants (Vitrasert®) Surgically implanted directly into the eye.
                                                                                   Lupus Retinopathy
Fomivirsen (Vitravene®)             A shot directly into the eye.




                        Sheathing of Blood Vessels




                                                                                                       37
Lupus Choroidopathy
                                           Lupus Retinopathy and Optic Neuropathy




                      Systemic Lupus


                      Three forms of lupus are known
                               • Systemic lupus erythematosus
                               • Cutaneous lupus
                               • Drug-induced lupus


                      A multisystem autoimmune disorder
                      that commonly affects women of
                      childbearing age (women>>>men)

                      Common findings include malar rash,rash,
                      arthritis,
                      arthritis, oral ulcers, renal disease,
                      hematological,
                      hematological, seizures and psychosis,
                      pulmonary and GI                                           Spot”
                                                                           “Roth Spot”
       Iritis




                                                                                         38
                                                                            Laboratory Testing in Systemic Lupus Erythematosus
Systemic Lupus Erythematosus
                                                                                   • Complete Blood Count (CBC)
    • Lupus is more common in the African American,
      Asian and native American populations                                        • Platelet count

    • Approximately 15 % of patients with Lupus will have retinal disease          • Erythrocyte Sedimentation Rate (ESR) - Westergren

    • Retinopathy can be associated with central nervous system                    • C-Reactive Protein (CRP)
      complications such as cerebritis
                                                                                   • Antinuclear antibody (ANA) – screening test
 Clinical Retinal Features
                                                                                   • Anti-n DNA ( ordered when ANA is positive )
         • Cotton Wool Spots
         • Retinal Hemorrhages                                                     • Urinalysis
           Vaso-occlusive
         • Vaso-occlusive disease
         • Frosted branch periphlebitis                                            • Complement ( C3 and C4 ) serum levels




                                                                                                           Scleritis




                                                                                                                                         39
                                                  Feature                     Episcleritis                Scleritis

                                                     Number of patients         37                          97

                                                     Age                        45                          51

                                                     Gender                     30%                         29%       men
                                                                                70%                         71%       women

                                                     Race                       white 84%                   white 79%

                                                     Bitlateral                 49%                         51%

                                                     Systemic disease           RA                          18% RA
Scleral thinning and scleromalacia perforans
                                                                Scleritis:
                                               Episcleritis and Scleritis: Clinical Features and Treatment Results. Doug Jabs, et al.
                                               Ophthalmology 2000; 130:469-476.




                                                                           Metastatic”
                                                                          “Metastatic” Choroidal Tumor




                                                                                                                                        40
Choroidal Metastasis




                       Metastatic Tumors


                             • Lung cancer - #1 cause of death in males
                             • Breast cancer - #1 cause of death in females
                             • Prostate cancer – most common cancer in men
                                 (More common and more aggressive in African Americans)

                             • Colon cancer – 3rd most common in men, 2nd most common in women
                             • Melanoma – skin most common site of cancer development
                                 (Males = trunk) (Females = extremities)

                             • Ovarian cancer – disease of postmenopausal women
                             • Pancreatic cancer – usually >65 years old, wt. loss, jaundice, anorexia
                             • Uterine cancer – disease of postmenopausal women
                                 (abnormal vaginal discharge or bleeding)




                                                                                                         41
42
Metastatic Tumors

    • Choroidal Metastasis is the most common ocular tumor
    • Most metastases found in the choroid are from the breast and lung
    • Only ~ 65 % of patients with a choroidal metastasis have been
        diagnosed with a form of systemic cancer

Ocular Presentation:
    • Dome-shaped lesion yellow to orange in color
    • Most often in the posterior pole but can be in iris and ciliary body
    • They may be solitary or multifocal
    • Bilateral in ~ 30 % of cases

Treatment:
    • Observation, external beam radiation, radioactive plaque therapy
                                           px’
    • The goal of treatment is to save the px’s vision for the remainder of life




                                                                                   43
                                                                                    Streaks:
                                                                            Angioid Streaks:

                                                                                • Alteration / break of the Retinal Pigment Epithelium (RPE),
                                                                                     Bruch’s membrane and Choriocapillaris
                                                                                • Patient is usually asymptomatic
                                                                                • Approximately 50% have associated systemic disease
                                                                                • Decreased vision is secondary to choroidal neovascularization
                                                                                     membrane(CNVM) or a streak through the fovea


                                                                            Etiology:

                                                                                • Pseudoxanthoma elasticum (85%)        Idiopathic
                                                                                • Ehlers Danlos syndrome                Lead Poisoning
                                                                                • Paget’s Disease
                                                                                • Sickle Cell Anemia




       Appearance:
Fundus Appearance:
                                                                                    High Myopia                                 Trauma
   • Bilateral gray – red – brown linear bands radiating in a spoke wheel
        pattern from the optic nerve
                        d’
   • May have a peau d’orange fundus temporal to the macula
                                                        spots”
   • May have peripheral round lesions caled “ salmon spots”
   • Possibility of Optic nerve head drusen


Differential Diagnosis:
             Diagnosis:                           Systems:
                                         Affected Systems:

   • Myopia (lacquer cracks)             • Cardiovascular
   • Choroidal Rupture                            HTN
                                                  Angina Pectoris
                                                  Mitral Valve Prolapse
                                         • Skin                                  • Lacquer Cracks                         • Choroidal Rupture
                                         • Gastrointestinal




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                                                    Treatment:
                                                    Treatment: Angioid Streaks
Initial Presentation                3 weeks later
                                                       • Focal laser if (CNVM) is present
                                                       • Management of any underlying systemic disease


                                                           up:
                                                    Follow up:

                                                       • Twice a year with a dilated fundus examination
                                                       • Amsler Grid testing (~3 x week)
               • Choroidal Ruture




                 The End !!!!

              Any Questions ????

              pizzimen@nova.edu

               cpelino@pco.edu




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