Carrie Wright
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Transcript Carrie Wright
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
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
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
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
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
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.