• It is characterized by recurrent venous or arterial thrombotic events and/or recurrent pregnancy complications.
• AFAS; It can be primary, but it can also develop on the basis of many connective tissue diseases (secondary), most commonly SLE.
• Antiphospholipid antibodies;
o Anticardiolipin antibody
o Anti 132 glycoprotein I
o Lupus anticoagulant
• These antibodies; They can also occur in cases of infection, malignancy, drug exposure, etc. However, these are generally different; They are IgM in nature, low titer, transient and rarely cause thrombosis. Therefore, for the diagnosis of AFAS, these antibodies must be shown to be positive at the end of 12 weeks.
Clinical Findings
• The most common clinical finding is venous thrombosis. Deep vein thrombosis is the most common venous thrombosis. Apart from this, pulmonary embolism, superficial thrombophlebitis and other thrombosis (Budd Chiari syndrome etc.) can be seen.
• Among arterial thrombosis, the most common picture is stroke. In addition, heart valve involvement, transient ischemic attack, myocardial ischemia, etc. may develop.
• Thrombosis is not the only cause of some neurological manifestations in the course of AFAS.
Neurological findings of unknown etiology; migraine, epilepsy, chorea, cerebellar ataxia, transverse myelopathy.
• Thrombocytopenia and autoimmune hemolytic anemia may develop in AFAS.
• Obstetric complications (eclampsia, preeclampsia) and fetal complications (fetal loss, prematurity) can be seen.
Laboratory Findings
• Anticardiolipin and anti-132 glycoprotein-1 antibodies are investigated by ELISA method, and lupus anticoagulant is investigated by aPTT or diluted Russel viper venom test.
• The concept of lupus anticoagulant is fundamentally wrong terminology. Because; Although lupus anticoagulant prolongs aPTT in vitro, it tends to thrombosis in vivo.
• In order to show the presence of lupus anticoagulant, the mixing test is performed first, that is, normal plasma is added to the serum sample. However, aPTT does not return to normal. Then phospholipid is added to this mixture and aPTT returns to normal.
• In the presence of antiphospholipid antibodies, the VDRL test (syphilis) may be false positive.
Diagnosis
Full criteria for antiphospholipid antibody syndrome
Clinical criteria
1) Vascular thrombosis
* thrombosis In any tissue or organ; arterial, venous and small vessel
2) Pregnancy morbidity
* Loss of at least 1 fetus after the week 10 or
* at least 3 consecutive spontaneous abortions before the week 10 or
* at least 1 premature birth (due to eclampsia, preeclampsia) before the week 34
Laboratory criteria (At least 2 times with an interval of at least 12 weeks)
*Anticardiolipin antibody or
*Anti B2 glycoprotein 1 or
*Lupus anticoagulant
At least one clinical and at least one laboratory criterion is required for diagnosis.
Prophylaxis and Treatment
Primary prophylaxis
• Aspirin and hydroxychloroquine are given to high-risk patients who have not experienced any thrombotic events.
Treatment
• Anticoagulant, steroid, IVIG/plasmapheresis
Secondary prophylaxis
• Coumadin ± aspirin should be given to patients after the first thrombotic attack.
• Pregnancy morbidity is prevented with heparin + aspirin.