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The Neuro-ophthalmic and The Neuro-ophthalmic and Retinal Manifestations of Retinal Manifestations of Antiphospholipid Antiphospholipid Antibody Syndrome Antibody Syndrome Carrie Wright, O.D. Carrie Wright, O.D. Julie Ferguson, O.D. Julie Ferguson, O.D. Steven J. Grondalski, Steven J. Grondalski, O.D. O.D.

The Neuro-ophthalmic and Retinal Manifestations of Antiphospholipid Antibody Syndrome Carrie Wright, O.D. Julie Ferguson, O.D. Steven J. Grondalski, O.D

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Page 1: The Neuro-ophthalmic and Retinal Manifestations of Antiphospholipid Antibody Syndrome Carrie Wright, O.D. Julie Ferguson, O.D. Steven J. Grondalski, O.D

The Neuro-ophthalmic and The Neuro-ophthalmic and Retinal Manifestations ofRetinal Manifestations of

Antiphospholipid Antiphospholipid Antibody SyndromeAntibody Syndrome

Carrie Wright, O.D.Carrie Wright, O.D.

Julie Ferguson, O.D.Julie Ferguson, O.D.

Steven J. Grondalski, O.D.Steven J. Grondalski, O.D.

Page 2: The Neuro-ophthalmic and Retinal Manifestations of Antiphospholipid Antibody Syndrome Carrie Wright, O.D. Julie Ferguson, O.D. Steven J. Grondalski, O.D

AbstractAbstractA case of acute, recurrent amaurosis A case of acute, recurrent amaurosis

fugax, Hollenhorst plaque, bilateral cotton fugax, Hollenhorst plaque, bilateral cotton wool spots, bilateral retinal hemorrhages, wool spots, bilateral retinal hemorrhages, and a seven year history of gangrenous and a seven year history of gangrenous digits shows dramatic improvement digits shows dramatic improvement within four days of intravenous steroids.within four days of intravenous steroids.

Page 3: The Neuro-ophthalmic and Retinal Manifestations of Antiphospholipid Antibody Syndrome Carrie Wright, O.D. Julie Ferguson, O.D. Steven J. Grondalski, O.D

Clinical PresentationClinical Presentation

49 yo AA male 49 yo AA male C/O C/O AcuteAcute Vision lossVision loss

Description: Description: “I see total darkness out of my “I see total darkness out of my right eye”right eye”

Location:Location: ODOD Onset:Onset: 3 weeks3 weeks Duration:Duration: 15-20 minutes15-20 minutes Last occurrence:Last occurrence: 30 minutes prior to appointment30 minutes prior to appointment Frequency:Frequency: 3x per day3x per day

Page 4: The Neuro-ophthalmic and Retinal Manifestations of Antiphospholipid Antibody Syndrome Carrie Wright, O.D. Julie Ferguson, O.D. Steven J. Grondalski, O.D

AssociationsAssociations Numbness on face L sideNumbness on face L side

IntermittentIntermittent 3 weeks3 weeks

Gangrene tips of digitsGangrene tips of digits R and L handR and L hand 7 yrs7 yrs

Additional concernsAdditional concerns::

PosturalPostural Upon waking while lying down – no visionUpon waking while lying down – no vision Lying on right side – no visionLying on right side – no vision

Page 5: The Neuro-ophthalmic and Retinal Manifestations of Antiphospholipid Antibody Syndrome Carrie Wright, O.D. Julie Ferguson, O.D. Steven J. Grondalski, O.D

Gangrenous fingersGangrenous fingers

Page 6: The Neuro-ophthalmic and Retinal Manifestations of Antiphospholipid Antibody Syndrome Carrie Wright, O.D. Julie Ferguson, O.D. Steven J. Grondalski, O.D

Additional Systemic FindingsAdditional Systemic Findings

(+) (+) Weight lossWeight loss About 15 pounds in 3 weeksAbout 15 pounds in 3 weeks

(+) (+) Jaw claudicationJaw claudication Jaw felt tired while eating donut 4 days priorJaw felt tired while eating donut 4 days prior

(+) (+) Muscle weaknessMuscle weakness

(+)(+) General malaise General malaise for about 7 years for about 7 years

(-) Scalp tenderness(-) Scalp tenderness

Page 7: The Neuro-ophthalmic and Retinal Manifestations of Antiphospholipid Antibody Syndrome Carrie Wright, O.D. Julie Ferguson, O.D. Steven J. Grondalski, O.D

Ocular historyOcular history

Initial visit to VAMCInitial visit to VAMC No surgeries, lasers, injuries to eyesNo surgeries, lasers, injuries to eyes

Family Ocular historyFamily Ocular history (+) grandmother has glaucoma(+) grandmother has glaucoma

Page 8: The Neuro-ophthalmic and Retinal Manifestations of Antiphospholipid Antibody Syndrome Carrie Wright, O.D. Julie Ferguson, O.D. Steven J. Grondalski, O.D

Medical HistoryMedical History

HematuriaHematuria Red blood cells in the urineRed blood cells in the urine Abnormal bleeding in gI tractAbnormal bleeding in gI tract

Arterial thrombosisArterial thrombosis Ischemic Necrosis of DigitsIschemic Necrosis of Digits Olecranon bursitisOlecranon bursitis

Inflammation to bursaInflammation to bursa Secondary to repetitive trauma or infectionSecondary to repetitive trauma or infection

HyperlipidemiaHyperlipidemia ImpotenceImpotence Coronary Artery Coronary Artery

DiseaseDisease Arterial embolism Arterial embolism Last HgA1C was Last HgA1C was

5.3%5.3%

Page 9: The Neuro-ophthalmic and Retinal Manifestations of Antiphospholipid Antibody Syndrome Carrie Wright, O.D. Julie Ferguson, O.D. Steven J. Grondalski, O.D

MedicationsMedications

AspirinAspirin Mometasone FuroateMometasone Furoate LisinoprilLisinopril SimvastatinSimvastatin SorbitolSorbitol WarfarinWarfarin AlbuterolAlbuterol HydroxyzineHydroxyzine Metoprolol tartrateMetoprolol tartrate

AlendronateAlendronate FlunisolideFlunisolide GabapentinGabapentin MorphineMorphine HydrocodoneHydrocodone Docusate NaDocusate Na HydrochlorothiazideHydrochlorothiazide MultivitaminMultivitamin VardenafilVardenafil

Page 10: The Neuro-ophthalmic and Retinal Manifestations of Antiphospholipid Antibody Syndrome Carrie Wright, O.D. Julie Ferguson, O.D. Steven J. Grondalski, O.D

Ocular examinationOcular examination

VA without correctionVA without correction ODOD 20/2020/20 OSOS 20/2020/20

Pupils, EOMs, Confrontations were normalPupils, EOMs, Confrontations were normal Anterior segment unremarkableAnterior segment unremarkable

IOP by Goldmann IOP by Goldmann ODOD 11 mmHg11 mmHg OSOS 10 mmHg10 mmHg

Page 11: The Neuro-ophthalmic and Retinal Manifestations of Antiphospholipid Antibody Syndrome Carrie Wright, O.D. Julie Ferguson, O.D. Steven J. Grondalski, O.D

ODOD

ONHONH CDRCDR 0.3 0.3 (+) hyperemia(+) hyperemia (+) blurred superior margins(+) blurred superior margins

VesselsVessels 1/4 A / V 1/4 A / V (+) multiple cotton wool spots x (+) multiple cotton wool spots x

2525 Superior arcadeSuperior arcade NasalNasal Inferior arcadeInferior arcade

(+) Flame hemorrhages x 3(+) Flame hemorrhages x 3 superior arcade x 2superior arcade x 2 inferior to ONH x 1inferior to ONH x 1

Page 12: The Neuro-ophthalmic and Retinal Manifestations of Antiphospholipid Antibody Syndrome Carrie Wright, O.D. Julie Ferguson, O.D. Steven J. Grondalski, O.D

OSOS

CDRCDR 0.20.2(+) hyperemia superior > inferior(+) hyperemia superior > inferior(+) blurred margins 9-11:00 (+) blurred margins 9-11:00 (+) obscured arteriole at 11:00 and (+) obscured arteriole at 11:00 and

12:0012:00

1/4 A /V ratio 1/4 A /V ratio (+) flame shaped hemorrhage x 1(+) flame shaped hemorrhage x 1(+) Cotton wool x 25-28(+) Cotton wool x 25-28

superiorsuperiorinferiorinferiorpapillomacular bundlepapillomacular bundle

(+) Hollenhorst plaque O.S.(+) Hollenhorst plaque O.S.

Page 13: The Neuro-ophthalmic and Retinal Manifestations of Antiphospholipid Antibody Syndrome Carrie Wright, O.D. Julie Ferguson, O.D. Steven J. Grondalski, O.D

Hollenhorst PlaqueHollenhorst Plaque

Page 14: The Neuro-ophthalmic and Retinal Manifestations of Antiphospholipid Antibody Syndrome Carrie Wright, O.D. Julie Ferguson, O.D. Steven J. Grondalski, O.D

Retinal EvaluationRetinal Evaluation

Page 15: The Neuro-ophthalmic and Retinal Manifestations of Antiphospholipid Antibody Syndrome Carrie Wright, O.D. Julie Ferguson, O.D. Steven J. Grondalski, O.D

Assessment Assessment

1. Amaurosis fugax OD1. Amaurosis fugax OD No bruit on auscultationNo bruit on auscultation

2. Hollenhorst plaque OS2. Hollenhorst plaque OS No bruit on auscultationNo bruit on auscultation

(+) Hx of multiple episodes of thrombosis(+) Hx of multiple episodes of thrombosis (+) Anticoagulation therapy with Coumadin; ASA(+) Anticoagulation therapy with Coumadin; ASA

3. Bilateral Cotton Wools with hemorrhages3. Bilateral Cotton Wools with hemorrhages No risk factors for HIVNo risk factors for HIV Retinal vasculitis OURetinal vasculitis OU Multiple episode of thrombosisMultiple episode of thrombosis Ischemia to digits of both handsIschemia to digits of both hands

4. Papillitis OS>OD4. Papillitis OS>OD

Page 16: The Neuro-ophthalmic and Retinal Manifestations of Antiphospholipid Antibody Syndrome Carrie Wright, O.D. Julie Ferguson, O.D. Steven J. Grondalski, O.D

Plan ?Plan ?

Digit and Posterior Segment Photos takenDigit and Posterior Segment Photos taken

Lab tests orderedLab tests ordered CRP/ESRCRP/ESR Homocysteine levelHomocysteine level CDC with differentialCDC with differential

MRI/MRA orderedMRI/MRA ordered Head and neckHead and neck

ER admit into hospitalER admit into hospital Start IV steroids with rheumatology consultStart IV steroids with rheumatology consult

Neurology Consult obtainedNeurology Consult obtained

Page 17: The Neuro-ophthalmic and Retinal Manifestations of Antiphospholipid Antibody Syndrome Carrie Wright, O.D. Julie Ferguson, O.D. Steven J. Grondalski, O.D

MRA - normalMRA - normal

Page 18: The Neuro-ophthalmic and Retinal Manifestations of Antiphospholipid Antibody Syndrome Carrie Wright, O.D. Julie Ferguson, O.D. Steven J. Grondalski, O.D

MRI - normalMRI - normal

Page 19: The Neuro-ophthalmic and Retinal Manifestations of Antiphospholipid Antibody Syndrome Carrie Wright, O.D. Julie Ferguson, O.D. Steven J. Grondalski, O.D

Differential DiagnosisDifferential Diagnosis

Retinal VasculitisRetinal Vasculitis

Retinal ArteritisRetinal Arteritis Behcet’s disease, Polyarteritis nodosa, Collagen Vascular Behcet’s disease, Polyarteritis nodosa, Collagen Vascular

disease, Associated vasculitis, Toxoplasmosis, Eales disease, Associated vasculitis, Toxoplasmosis, Eales disease, HIV, Antiphospholipid Antibody Syndrome, disease, HIV, Antiphospholipid Antibody Syndrome, SyphilisSyphilis

Retinal PhlebitisRetinal Phlebitis Sarcoidosis, Tuberculosis, Syphilis, MS, Pars planitis, Eales Sarcoidosis, Tuberculosis, Syphilis, MS, Pars planitis, Eales

disease, Antiphospholipid Antibody Syndrome, HIV, Frosted disease, Antiphospholipid Antibody Syndrome, HIV, Frosted Branch angitisBranch angitis

Page 20: The Neuro-ophthalmic and Retinal Manifestations of Antiphospholipid Antibody Syndrome Carrie Wright, O.D. Julie Ferguson, O.D. Steven J. Grondalski, O.D

Additional Work-upAdditional Work-up

Rheumatology consultRheumatology consult

Lab tests orderedLab tests ordered RPR, FTA-Abs, ACE, ANA, RF, LA, INRRPR, FTA-Abs, ACE, ANA, RF, LA, INR

Bilateral Carotid UltrasoundBilateral Carotid Ultrasound

Temporal Artery BiopsyTemporal Artery Biopsy

Page 21: The Neuro-ophthalmic and Retinal Manifestations of Antiphospholipid Antibody Syndrome Carrie Wright, O.D. Julie Ferguson, O.D. Steven J. Grondalski, O.D

Lab ResultsLab ResultsTestTest ScoreScore High/LowHigh/Low NormalNormal

PTPT 40.240.2 HH 12.2-15.112.2-15.1

APTTAPTT 51.251.2 HH 19.5-38.519.5-38.5

CRPCRP 14.1714.17 HH .01-.82.01-.82

ESRESR 112112 HH 0-150-15

RFRF NEGNEG

RPRRPR No rxnNo rxn

ANAANA No RxnNo Rxn

ACEACE LowLow

INRINR >4>4

FTA-ABSFTA-ABS NEGNEG

Page 22: The Neuro-ophthalmic and Retinal Manifestations of Antiphospholipid Antibody Syndrome Carrie Wright, O.D. Julie Ferguson, O.D. Steven J. Grondalski, O.D

INR for Past 2 YearsINR for Past 2 Years↓ INR 5.6 5/30/07

Page 23: The Neuro-ophthalmic and Retinal Manifestations of Antiphospholipid Antibody Syndrome Carrie Wright, O.D. Julie Ferguson, O.D. Steven J. Grondalski, O.D

New differential New differential diagnosisdiagnosis

Hypercoagulative StateHypercoagulative State Antiphospholipid Antibody SyndromeAntiphospholipid Antibody Syndrome

AtherosclerosisAtherosclerosis Ordered Ultrasound of carotid, which showed No stenosisOrdered Ultrasound of carotid, which showed No stenosis

Giant Cell ArteritisGiant Cell Arteritis Patient is young with history of occlusive disease and no Patient is young with history of occlusive disease and no

headacheheadache

Page 24: The Neuro-ophthalmic and Retinal Manifestations of Antiphospholipid Antibody Syndrome Carrie Wright, O.D. Julie Ferguson, O.D. Steven J. Grondalski, O.D

Temporal Artery BiopsyTemporal Artery Biopsy

NegativeNegative

Page 25: The Neuro-ophthalmic and Retinal Manifestations of Antiphospholipid Antibody Syndrome Carrie Wright, O.D. Julie Ferguson, O.D. Steven J. Grondalski, O.D

ODODInitial 4 days 2 Initial 4 days 2

weeksweeks

Page 26: The Neuro-ophthalmic and Retinal Manifestations of Antiphospholipid Antibody Syndrome Carrie Wright, O.D. Julie Ferguson, O.D. Steven J. Grondalski, O.D

OSOSInitial 4 days 2 weeksInitial 4 days 2 weeks

Page 27: The Neuro-ophthalmic and Retinal Manifestations of Antiphospholipid Antibody Syndrome Carrie Wright, O.D. Julie Ferguson, O.D. Steven J. Grondalski, O.D

Antiphospholipid Antibody Antiphospholipid Antibody SyndromeSyndrome

Also known AsAlso known As1.1. Hughes SyndromeHughes Syndrome

2.2. Sticky blood SyndromeSticky blood Syndrome

3.3. Lupus Anticoagulant (LAC) - misnomerLupus Anticoagulant (LAC) - misnomer

4.4. APSAPS

Two TypesTwo Types1.1. Primary (PAPS)Primary (PAPS)

Without a secondary disease processWithout a secondary disease process

2.2. SecondarySecondary

Associated with autoimmune or other systemic Associated with autoimmune or other systemic disease condition disease condition

Most commonly systemic lupus erythematosusMost commonly systemic lupus erythematosus

Page 28: The Neuro-ophthalmic and Retinal Manifestations of Antiphospholipid Antibody Syndrome Carrie Wright, O.D. Julie Ferguson, O.D. Steven J. Grondalski, O.D

Antiphospholipid Antibody SyndromeAntiphospholipid Antibody Syndrome

Hypercoagulative State Hypercoagulative State Increased risk of thrombosisIncreased risk of thrombosis

Autoantibodies present-directed against phospholipidsAutoantibodies present-directed against phospholipids Healthy young individualsHealthy young individuals Tend to increase with ageTend to increase with age Tend to increase in patients with SLETend to increase in patients with SLE

If an If an asymptomaticasymptomatic patient has antibodies to patient has antibodies to phospholipids (even in association with SLE), they are phospholipids (even in association with SLE), they are not considered to have APSnot considered to have APS So, what is the criteria for APS?So, what is the criteria for APS?

Page 29: The Neuro-ophthalmic and Retinal Manifestations of Antiphospholipid Antibody Syndrome Carrie Wright, O.D. Julie Ferguson, O.D. Steven J. Grondalski, O.D

Diagnostic criterion for APSDiagnostic criterion for APS (Antiphospholipid Syndrome)(Antiphospholipid Syndrome)

Pt must meet one of two clinical criteriaPt must meet one of two clinical criteria Complications with pregnancyComplications with pregnancy

Including fetal loss, prematurity, and stillbirthIncluding fetal loss, prematurity, and stillbirth

One or more clinical episodes with Vascular Thrombosis One or more clinical episodes with Vascular Thrombosis confirmed with imaging or histopathyconfirmed with imaging or histopathy

Pt must also meet one of two laboratory criteriaPt must also meet one of two laboratory criteria Anticardiolipin antibody of immunoglobin G on 2 or Anticardiolipin antibody of immunoglobin G on 2 or

more occasions at least 6 weeks apartmore occasions at least 6 weeks apart

Lupus anticoagulant on 2 or more occasions at least 6 Lupus anticoagulant on 2 or more occasions at least 6 weeks apartweeks apart

Antibodies block phospholipid surface which is Antibodies block phospholipid surface which is important for coagulationimportant for coagulation

New England Journal of Medicine,New England Journal of Medicine, March 2002 March 2002

Page 30: The Neuro-ophthalmic and Retinal Manifestations of Antiphospholipid Antibody Syndrome Carrie Wright, O.D. Julie Ferguson, O.D. Steven J. Grondalski, O.D

Clinical Features of APSClinical Features of APS

Deep Vein Thrombosis > Arterial ThrombosisDeep Vein Thrombosis > Arterial Thrombosis The brain is a common site for arterial The brain is a common site for arterial

thrombosis in APS thrombosis in APS Stroke and TIAs make up 50% of all arterial emboliStroke and TIAs make up 50% of all arterial emboli

Pulmonary EmboliPulmonary Emboli ThrombocytopeniaThrombocytopenia Hemolytic AnemiaHemolytic Anemia Livedo ReticularisLivedo Reticularis

Narrowed or constricted blood vesselsNarrowed or constricted blood vessels

Page 31: The Neuro-ophthalmic and Retinal Manifestations of Antiphospholipid Antibody Syndrome Carrie Wright, O.D. Julie Ferguson, O.D. Steven J. Grondalski, O.D

Ocular ManifestationsOcular Manifestations

Ocular retinal findings are present in Ocular retinal findings are present in

80% of patients with APS80% of patients with APS

TIA and TVLTIA and TVL Transient diplopiaTransient diplopia Ischemic optic neuropathyIschemic optic neuropathy Retinal vascular occlusionRetinal vascular occlusion Peripheral proliferative retinopathyPeripheral proliferative retinopathy

According to According to Optometry: Journal ofOptometry: Journal of the AOA the AOA

Page 32: The Neuro-ophthalmic and Retinal Manifestations of Antiphospholipid Antibody Syndrome Carrie Wright, O.D. Julie Ferguson, O.D. Steven J. Grondalski, O.D

Manifestations pertinent Manifestations pertinent to our caseto our case

HematuriaHematuria Gangrene (arterial thrombosis)Gangrene (arterial thrombosis) Muscle weaknessMuscle weakness TIATIA

Page 33: The Neuro-ophthalmic and Retinal Manifestations of Antiphospholipid Antibody Syndrome Carrie Wright, O.D. Julie Ferguson, O.D. Steven J. Grondalski, O.D

Catastrophic Antiphospholipid Catastrophic Antiphospholipid

AntibodyAntibody Multiple simultaneous vascular occlusions Multiple simultaneous vascular occlusions

throughout the body throughout the body

Mortality is 50% Mortality is 50% Usually with multi-organ failureUsually with multi-organ failure

Usually affects small vessels>large vessels Usually affects small vessels>large vessels

Must affect at least three different organ systems Must affect at least three different organ systems to be considered catastrophicto be considered catastrophic

Page 34: The Neuro-ophthalmic and Retinal Manifestations of Antiphospholipid Antibody Syndrome Carrie Wright, O.D. Julie Ferguson, O.D. Steven J. Grondalski, O.D

TreatmentTreatment

““Management of Antiphospholipid Antibody Syndrome”Management of Antiphospholipid Antibody Syndrome”

Antiphospholipid antibodies and venous thrombosisAntiphospholipid antibodies and venous thrombosis Long term anticoagulantLong term anticoagulant

Warfarin until INR = 2-3Warfarin until INR = 2-3

Antiphospholipid antibodies and first time strokeAntiphospholipid antibodies and first time stroke Moderate intensity Warfarin or aspirinModerate intensity Warfarin or aspirin

Asymptomatic patientAsymptomatic patient Jury is still out !!!!Jury is still out !!!!

JAMA, 2006JAMA, 2006

Page 35: The Neuro-ophthalmic and Retinal Manifestations of Antiphospholipid Antibody Syndrome Carrie Wright, O.D. Julie Ferguson, O.D. Steven J. Grondalski, O.D

ReferencesReferences1.1. Levine JS, Branch DW, Rauch J. The Antiphospholipid Antibody Levine JS, Branch DW, Rauch J. The Antiphospholipid Antibody

Syndrome. Syndrome. N Engl J MedN Engl J Med 2002; 346 (10): 752-63. 2002; 346 (10): 752-63.

2.2. Tomasini DN, Segu B. Systemic Considerations in Bilateral Central Tomasini DN, Segu B. Systemic Considerations in Bilateral Central Retinal Vein Occlusion. Retinal Vein Occlusion. OptometryOptometry 2007; 78; 402-408. 2007; 78; 402-408.

3.3. Lim W, Crowther MA, Eikelboom JW. Management of Lim W, Crowther MA, Eikelboom JW. Management of Antiphospholipid Antibody Syndrome-At Systematic Review. Antiphospholipid Antibody Syndrome-At Systematic Review. JAMAJAMA, , March 1, 2006; 295 (9): 1050-1057March 1, 2006; 295 (9): 1050-1057

4.4. Myones BL, McCurdy D. Antiphopholipid Antibody Syndrome. Myones BL, McCurdy D. Antiphopholipid Antibody Syndrome. EmedicineEmedicine. Oct 26, 2004.. Oct 26, 2004.

5.5. Kunimoto DY, Kanitkar KD, Makar MS. The Wills Eye Manual, 4Kunimoto DY, Kanitkar KD, Makar MS. The Wills Eye Manual, 4thth ed. ed. Philadelphia: Lippincott Williams and Wilkins; 2004. Philadelphia: Lippincott Williams and Wilkins; 2004.