Antiphosholipid antibody syndrome


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Antiphosholipid antibody syndrome

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Antiphosholipid antibody syndrome

  1. 1. ANTIPHOSPHOLIPID ANTIBODY SYNDROME ` Antiphospholipid Antibody syndrome Venous thromboembolism during pregnancy DEFINITION: It is a condition in which antibodies (lupus anticoagulant and anti cardiolipins antibodies) are formed against vascular endothelium and plattlets leading to vasoconstriction, thrombosis, placental infarctions and fetal loss PATHOGENESIS: Antiphosholipid antibodies (lupus anticoagulant and anti cardiolipins antibodies) relative excess of thromboxane E2 (vasodilator and prevent plattlets aggregation) reduces the release of hCG (vasoconstrictor substance) blocks the action of prostacyclin inhibits trophoplastic invasion vasculopathy of Spiral Arterioles Thrombosis uteroplacental insufficiency Oligohydraminos Fetal Hypoxia IUGR Fetal loss A-MOWAFY 2013 1
  2. 2. ANTIPHOSPHOLIPID ANTIBODY SYNDROME ` EFFECT OF ANTIPHOSPHOLIPID ANTIBODIES ON PREGNANCY: 1. 2. 3. 4. 5. 6. 7. Recurrent pregnancy loss (typically at or beyond 10 weeks) IUGR IUFD Early onset pre-eclampsia (before 20 weeks) Placental abruption Preterm labour Occasionally infertility DIAGNOSIS: Criteria for diagnosis should include at least one clinical data + one laboratory data  Clinical criteria: I. Pregnancy morbidity: i.Any unexplained fetal death of a morphologically normal fetus at or beyond 10 weeks (normality is determined by direct examination or ultrasound) ii.Any birth of a morphologically normal neonate before 34 weeks due to SPET or severe placental insufficiency iii.At least 3 consecutive spontaneous abortions before 10 weeks after exclusion of maternal, anatomical, hormonal and chromosomal causes (of both parents) II. Vascular thrombosis: past or current history of: i.Recurrent DVT ii.Peripheral arterial gangrene iii.Cerebro-vascular stroke iv.Coronary thrombosis v.Pulmonary hypertension III. Associated immune and connective tissue disease: i.SLE ii. Thrombocytopenia IV. Persistent false positive Wasserman’s test V. Infertility  Laboratory criteria: I. Positive titres of moderate to high dilution of anti-cardiolipin antibody at least 12 weeks apart (IgG, IgM) II. Anti-β2 glycoprotein antibody III. Lupus anti-coagulant antibody (Russell’s Viber Venom test) A-MOWAFY 2013 2
  3. 3. ANTIPHOSPHOLIPID ANTIBODY SYNDROME ` MANAGEMENT:  Lines of management: I. II. III. IV. V. VI. Pre-conception counselling Throboprophylaxis Corticosteroid therapy Immunologic therapy Prevention of complications Post-partum care I. PRE-CONCEPTION COUNSELLING:  Counselling the patient about the possible risk and pregnancy outcome  Patients with antiphospholoipid antibody syndrome secondary to renal disease, SLE, thrombocytopenia, and hemolytic anemia need a specialized care  Pre-conceptional correction of anemia, thrombocytopenia ….etc II. THROBOPROPHYLAXIS:  Low dose Aspirin: Aspirin (60 – 80 mg) tablet once daily  Heparin: Onset I. Unfractionated heparin → UFH “Cal-heparin” II. Low molecular weight heparin → LMWH “Clexan” Within 10 minutes Half-life 6 hours Types Doses I.     II. Monitoring A-MOWAFY 2013     1. 2. 3. Unfractionated heparin Normal body weight → 5000 IU/day Body weight ˂ 50 kg → 2500 IU/day Body weight ˃ 90 kg → 5000 IU/12 h High prophylactic dose→ 100 IU/kg/12h Low molecular weight heparin Normal body weight → 40 mg/day Body weight ˂ 50 kg → 20 mg /day Body weight ˃ 90 kg → 40 mg / 12h High prophylactic dose→ I mg/kg/12h Activated partial thromboplastin aPPT twice weekly Plattlets concentration monthly Bone mineral density every 3 months 3
  4. 4. ANTIPHOSPHOLIPID ANTIBODY SYNDROME ` Advantages Drawbacks Anti-dot 1. No effect on the fetus ( does not cross placental blood barrier ) 2. Not secreted on milk ( no effect on lactation ) N.B : advantages of LMWH over UFH o Fewer side effect o Prolonged half-life o Greater inhibitory effect on factor X o Prophylactic dose once daily o No need for continuous laboratory monitoring  Increase bleeding tendency  Osteoporosis ( improved by adding calcium , hence name “ Cal-heparin “  Fat necrosis at site of injection  Alopecia Protamine sulphate 1mg/100IU N.B: Epidural anesthesia should be avoided in patients under heparin therapy III. CORTICOSTEROID THERAPY:  Prednisone ( ˃40 mg/day) in combination with low dose aspirin  Complications include: cushenoid manifestation, induced DM, predisposition to infections IV. IMMUNOLOGIC THERAPY:  Indications : a. Failure of 1st line therapy b. Associated hypertensive disorders c. Associated IUGR  Immunoglobulins IV 0.4 gm/kg/daily for 5 days and repeated monthly  Immunosuppressive drugs; azathioprine, cyclosporins do not improve success rate A-MOWAFY 2013 4
  5. 5. ANTIPHOSPHOLIPID ANTIBODY SYNDROME ` V. PREVENTION OF COMPLICATIONS:  Continuous monitoring by: a. ANC weekly b. NST and ultrasound every 2 weeks c. Doppler ultrasound for early detection of uteroplacental insufficiency and IUGR  Corticosteroid therapy may be used for lung maturity but it is better to be avoided in heparinized patients  High risk patients should be fully anticoagulated with LMWH throughout the whole pregnancy  Low-dose aspirin alone is enough if no complications VI. POST-PARTUM CARE:  Thromboprophylaxis should be continued for first 6 weeks postpartum  Warfarin can be used to achieve INR values between 2.0 and 3.0 even in breast feeding woman  Estrogen-containing pills are contraindicated N.B: Postpartum examination of placenta is important In complicated cases the placenta appears of low weight, massive infarctions and intravascular thrombosis A-MOWAFY 2013 5