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Anti Phospholipid Syndrome

 Anti Phospholipid Syndrome (APS) research

THE MOSAIC OF APS

Additional interesting aspects of APS 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 APS, 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 APS is a multi-disciplinary disease.

Precipitating factors in 80 patients with catastrophic APS

 
 

No.

(%)

     

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

   
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*
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 APS 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 discontinuation

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.
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