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Facts and Figures
THE MOSAIC OF ANTIPHOSPHOLIPID SYNDROME (APS)
Additional interesting aspects of Antiphospholipid Syndrome (APS or APLS) raised by presenters included:
1. High titers of aCL in advanced coronary atherosclerosis (I. Stankulov). 2. The appearance of aCL often with myocardial infarction. 3. The 5% to 10% incidence of epilepsy in Antiphospholipid Syndrome (APS or APLS), related to the effect of aCL on the central nervous system. 4. The 29% of aCL positivity in sera
of patients (I. Kokareshkov) with multiple sclerosis.
All the above indicates that indeed Antiphospholipid Syndrome (APS or APLS) is a multi-disciplinary disease.
Precipitating factors in 80 patients with catastrophic Antiphospholipd Syndrome (CAPS)
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No. |
(%) |
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Infections |
12 |
(5) |
Respiratory tract |
6 |
(8) |
Cutanous |
5 |
(6) |
Urinary tract |
2 |
(3) |
Spesis |
1 |
(1) |
Gastrointestinal |
7 |
(9) |
Other |
10 |
(13) |
Surgery, trauma, and invasive procedures |
6 |
(8) |
Neoplasia |
6 |
(8) |
Anticoagulation withdrawal/low INR |
5 |
(6) |
Obstetric complications |
4 |
(5) |
SLE flares |
2 |
(3) |
Oral contraceptives |
28 |
(35) |
No factor identified |
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Criteria for the Diagnosis of Antiphospholipid Antibody Syndrome
Clinical |
Laboratory |
· Recurrent venous thrombosis
· Recurrent arterial thrombosis
· Recurrent fetal loss+
· Persistent thrombocytopenia
· Livedo reticularis
|
- IgG or IgM anticardiolipin (anti-B2-glycoprotein-I dependent) antibody (> 20 IU) ++
- Lupus anticoagulant*
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Patients must have at least one clinical and 1 laboratory finding and laboratory test
result must be positive on at least 2 occasions more than 3 months apart. + As defined by Branch and Silver. (At least 3 spontaneous abortions, fetal death,or
early neonatal death due to preterm delivery required because of fetal distress. )1 This may soon be supplanted by a direct test for antibody to B2-glycoprotein-I.
*Phospholipid-dependent screening test, such as activated partial thromboplastin time, must be abnormal.
Treatment
For recurrent manifestations of Antiphospholipid Syndrome (APS or APLS) anticoagulation is the treatment of choice. For treatment of recurrent fetal loss heparin and aspirin increases survival from 40% to 80% 25.
Recent Treatment Studies in Antiphospholipid Antibody Syndrome*
Condition |
Study |
Type |
Comparison |
Conclusion |
Comment |
Clot (all) |
Khamashta et al11 |
Retrospective |
Aspirin vs warfarin |
INR> 3 protective |
INR may be an invalid test |
Clot (stroke) |
Brey and Levine29 |
RCT |
Aspirin vs warfarin sodium, different warfarin doses |
In progress |
... |
Clot (venous) |
Ginsberg et al |
RCT |
Different warfarin doses |
Moderate dose sufficient |
15% recurrence at 6 mo after discontinuati on |
Pregnancy |
Cowchock et al |
RCT |
Aspirin + heparin vs Aspirin + Prednisone |
Aspirin + heparin better |
Small study |
Pregnancy |
Pregnancy Loss Study Group |
RCT |
Aspirin + heparin vs IVIG |
In progress |
... |
Pregnancy |
Kutteh |
RCT |
Aspirin vs heparin, different heparin doses |
Heparin better, low dose as effective as high dose |
Small study |
INR indicates international normalized ratio;RCT,
randomized controlled trial; and IVIG, intravenous immunoglobulin.
Page forms part of www.apls.tk, the information site on ANTIPHOSPHOLIPID SYNDROME (APS or ANTIPHOSPHOLIPID SYNDROME (APLS))
Medical Keywords: systemic antiphospholipid antibody syndrome, Antiphospholipid, Antiphospholipid Antibody Syndrome, Antiphospholipid Syndrome, APS, APLS, Hughes
Syndrome, Sticky Blood, Clotting Disorder, Stroke, TIA, PE, death, Antiphospholipid Antibody Syndrome, Antiphospholipid Syndrome, APS, APLS,
Hughes Syndrome, Sticky Blood, Clotting Disorder, Stroke, TIA, PE, death
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