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Antiphospholipid Antibody Syndrome (Other manifestations (Cardiac valvular…
Antiphospholipid Antibody Syndrome
Epidemiology
70-80% female
Caucasians
Primary or assoc w/ SLE
Pathogenesis
Ab. induced thrombosis?
Activate endothelial cells/platelets
Inhibit protein C activation
Promote tissue factor synth by leukocytes
Markers of another process?
Antiphospholipid Ab secondary to infxn or drugs usually do not experience thrombosis
Symptoms
Asymp aPL positivity
APS w/ vascular events
Venous clot
Arterial clot
Pregnancy-related clot
APS w/ pregnancy morbidity
No clotting
1+ unexplained death of normal fetus >10 wks
1+ premature births before 34 wks gest. b/o preeclampsia, eclampsia, or placenta insufficiency
3+ unexplained consecutive SAB <10 wks gest.
Catastrophic APS
3+ organs in short period
High mortality
Other manifestations
Cardiac valvular abnormalities
Mitral valve
Libman-Sacs Endocarditis
Thrombocytopenia (~30%)
Positive Coomb's (10-20%)
False positive VDRL
Seizures, TIA/CVA
Livedo reticularis (marbled red rash)
Renal system clots
Labs
Not great tests. Many false positives.
Repeat 12 wks apart to confirm
Specific labs
Lupus anticoagulant (DRVVT)
Anti-cardiolipin Ab
Anti-Beta 2 glycoprotein Ab
Treatment
Preventative
Hydroxychloroquine (for aPL pts w/ SLE)
Address other issues
OCPs
Tobacco, HTN, DM, HLP
ASA (no data supporting)
Pregnancy
ASA + Heparin
: improves fetal survival 80-90%
Treatment
Long-term anticoag
Warfarin
Catastrophic APS
Plasmaphoresis
Steroids
Immunosuppression
ABX