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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.
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.
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
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
Gangrenous fingersGangrenous fingers
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
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
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%
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
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
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
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.
Hollenhorst PlaqueHollenhorst Plaque
Retinal EvaluationRetinal Evaluation
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
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
MRA - normalMRA - normal
MRI - normalMRI - normal
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
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
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
INR for Past 2 YearsINR for Past 2 Years↓ INR 5.6 5/30/07
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
Temporal Artery BiopsyTemporal Artery Biopsy
NegativeNegative
ODODInitial 4 days 2 Initial 4 days 2
weeksweeks
OSOSInitial 4 days 2 weeksInitial 4 days 2 weeks
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
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?
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
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
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
Manifestations pertinent Manifestations pertinent to our caseto our case
HematuriaHematuria Gangrene (arterial thrombosis)Gangrene (arterial thrombosis) Muscle weaknessMuscle weakness TIATIA
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
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
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.