Download Antiphospholipid Antibody Syndrome

The Neuro-ophthalmic and
Retinal Manifestations of
Antiphospholipid
Antibody Syndrome
Carrie Wright, O.D.
Julie Ferguson, O.D.
Steven J. Grondalski, O.D.
Abstract
A case of acute, recurrent amaurosis
fugax, Hollenhorst plaque, bilateral cotton
wool spots, bilateral retinal hemorrhages,
and a seven year history of gangrenous
digits shows dramatic improvement
within four days of intravenous steroids.
Clinical Presentation
 49 yo AA male
C/O Acute Vision loss
 Description:
“I see total darkness out of my
right eye”
 Location:
 Onset:
 Duration:
 Last occurrence:
 Frequency:
OD
3 weeks
15-20 minutes
30 minutes prior to appointment
3x per day
 Associations
 Numbness on face L side
 Intermittent
 3 weeks
 Gangrene tips of digits
 R and L hand
 7 yrs
 Additional concerns:
 Postural
 Upon waking while lying down – no vision
 Lying on right side – no vision
Gangrenous fingers
Additional Systemic Findings
 (+) Weight loss
 About 15 pounds in 3 weeks
 (+) Jaw claudication
 Jaw felt tired while eating donut 4 days prior
 (+) Muscle weakness
 (+) General malaise for about 7 years
 (-) Scalp tenderness
Ocular history
 Initial visit to VAMC
 No surgeries, lasers, injuries to eyes
 Family Ocular history
 (+) grandmother has glaucoma
Medical History
 Hematuria
 Hyperlipidemia
 Red blood cells in the urine
 Impotence
 Abnormal bleeding in gI tract
 Coronary Artery
 Arterial thrombosis
Disease
 Ischemic Necrosis of Digits
 Arterial embolism
 Olecranon bursitis
 Last HgA1C was
 Inflammation to bursa
5.3%
 Secondary to repetitive trauma or infection
Medications

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

Aspirin
Mometasone Furoate
Lisinopril
Simvastatin
Sorbitol
Warfarin
Albuterol
Hydroxyzine
Metoprolol tartrate









Alendronate
Flunisolide
Gabapentin
Morphine
Hydrocodone
Docusate Na
Hydrochlorothiazide
Multivitamin
Vardenafil
Ocular examination
 VA without correction
 OD
 OS
20/20
20/20
 Pupils, EOMs, Confrontations were normal
 Anterior segment unremarkable
 IOP by Goldmann
 OD
 OS
11 mmHg
10 mmHg
OD

ONH
 CDR
0.3
 (+) hyperemia
 (+) blurred superior margins

Vessels
 1/4 A / V
 (+) multiple cotton wool spots x
25
 Superior arcade
 Nasal
 Inferior arcade
 (+) Flame hemorrhages x 3
superior arcade x 2
inferior to ONH x 1
OS
CDR
0.2
(+) hyperemia superior > inferior
(+) blurred margins 9-11:00
(+) obscured arteriole at 11:00 and
12:00
1/4 A /V ratio
(+) flame shaped hemorrhage x 1
(+) Cotton wool x 25-28
superior
inferior
papillomacular bundle
(+) Hollenhorst plaque O.S.
Hollenhorst Plaque
Retinal Evaluation
Assessment
1. Amaurosis fugax OD
 No bruit on auscultation
2. Hollenhorst plaque OS
 No bruit on auscultation
 (+) Hx of multiple episodes of thrombosis
 (+) Anticoagulation therapy with Coumadin; ASA
3. Bilateral Cotton Wools with hemorrhages
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

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No risk factors for HIV
Retinal vasculitis OU
Multiple episode of thrombosis
Ischemia to digits of both hands
4. Papillitis OS>OD
Plan ?
 Digit and Posterior Segment Photos taken
 Lab tests ordered
 CRP/ESR
 Homocysteine level
 CDC with differential
 MRI/MRA ordered
 Head and neck
 ER admit into hospital
 Start IV steroids with rheumatology consult
 Neurology Consult obtained
MRA - normal
MRI - normal
Differential Diagnosis
 Retinal Vasculitis
 Retinal Arteritis
 Behcet’s disease, Polyarteritis nodosa, Collagen Vascular
disease, Associated vasculitis, Toxoplasmosis, Eales
disease, HIV, Antiphospholipid Antibody Syndrome, Syphilis
 Retinal Phlebitis
 Sarcoidosis, Tuberculosis, Syphilis, MS, Pars planitis, Eales
disease, Antiphospholipid Antibody Syndrome, HIV, Frosted
Branch angitis
Additional Work-up
 Rheumatology consult
 Lab tests ordered
 RPR, FTA-Abs, ACE, ANA, RF, LA, INR
 Bilateral Carotid Ultrasound
 Temporal Artery Biopsy
Lab Results
Test
Score
High/Low
Normal
PT
APTT
CRP
ESR
RF
RPR
ANA
ACE
INR
FTA-ABS
40.2
51.2
14.17
112
NEG
No rxn
No Rxn
Low
>4
NEG
H
H
H
H
12.2-15.1
19.5-38.5
.01-.82
0-15
INR for Past 2 Years
↓ INR 5.6
5/30/07
New differential diagnosis
 Hypercoagulative State
 Antiphospholipid Antibody Syndrome
 Atherosclerosis
 Ordered Ultrasound of carotid, which showed No stenosis
 Giant Cell Arteritis
 Patient is young with history of occlusive disease and no
headache
Temporal Artery Biopsy
Negative
Initial
OD
4 days
2 weeks
Initial
OS
4 days
2 weeks
Antiphospholipid Antibody Syndrome

Also known As
1.
2.
3.
4.

Hughes Syndrome
Sticky blood Syndrome
Lupus Anticoagulant (LAC) - misnomer
APS
Two Types
1. Primary (PAPS)
Without a secondary disease process
2. Secondary
Associated with autoimmune or other systemic
disease condition
Most commonly systemic lupus erythematosus
Antiphospholipid Antibody Syndrome
 Hypercoagulative State
 Increased risk of thrombosis
 Autoantibodies present-directed against phospholipids
 Healthy young individuals
 Tend to increase with age
 Tend to increase in patients with SLE
 If an asymptomatic patient has antibodies to
phospholipids (even in association with SLE), they are
not considered to have APS
 So, what is the criteria for APS?
Diagnostic criterion for APS (Antiphospholipid Syndrome)
 Pt must meet one of two clinical criteria
 Complications with pregnancy
 Including fetal loss, prematurity, and stillbirth
 One or more clinical episodes with Vascular Thrombosis
confirmed with imaging or histopathy
 Pt must also meet one of two laboratory criteria
 Anticardiolipin antibody of immunoglobin G on 2 or more
occasions at least 6 weeks apart
 Lupus anticoagulant on 2 or more occasions at least 6
weeks apart
 Antibodies block phospholipid surface which is
important for coagulation
New England Journal of Medicine, March 2002
Clinical Features of APS
 Deep Vein Thrombosis > Arterial Thrombosis
 The brain is a common site for arterial
thrombosis in APS
 Stroke and TIAs make up 50% of all arterial emboli
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Pulmonary Emboli
Thrombocytopenia
Hemolytic Anemia
Livedo Reticularis
 Narrowed or constricted blood vessels
Ocular Manifestations
Ocular retinal findings are present in
80% of patients with APS
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TIA and TVL
Transient diplopia
Ischemic optic neuropathy
Retinal vascular occlusion
Peripheral proliferative retinopathy
According to Optometry: Journal of the AOA
Manifestations pertinent
to our case

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Hematuria
Gangrene (arterial thrombosis)
Muscle weakness
TIA
Catastrophic Antiphospholipid
Antibody
 Multiple simultaneous vascular occlusions
throughout the body
 Mortality is 50%
 Usually with multi-organ failure
 Usually affects small vessels>large vessels
 Must affect at least three different organ systems to
be considered catastrophic
Treatment
“Management of Antiphospholipid Antibody Syndrome”

Antiphospholipid antibodies and venous thrombosis

Long term anticoagulant


Antiphospholipid antibodies and first time stroke


Warfarin until INR = 2-3
Moderate intensity Warfarin or aspirin
Asymptomatic patient

JAMA, 2006
Jury is still out !!!!
References
1.
Levine JS, Branch DW, Rauch J. The Antiphospholipid Antibody
Syndrome. N Engl J Med 2002; 346 (10): 752-63.
2.
Tomasini DN, Segu B. Systemic Considerations in Bilateral Central
Retinal Vein Occlusion. Optometry 2007; 78; 402-408.
3.
Lim W, Crowther MA, Eikelboom JW. Management of
Antiphospholipid Antibody Syndrome-At Systematic Review. JAMA,
March 1, 2006; 295 (9): 1050-1057
4.
Myones BL, McCurdy D. Antiphopholipid Antibody Syndrome.
Emedicine. Oct 26, 2004.
5.
Kunimoto DY, Kanitkar KD, Makar MS. The Wills Eye Manual, 4th ed.
Philadelphia: Lippincott Williams and Wilkins; 2004.