Antiphospholipid Syndrome and Pregnancy

Obstetrics

Clinicals - History

Fact Explanation
Symptoms of pre-eclampsia Pre-eclampsia can occur due to recurrent thrombosis of the placental bed. Pregnant ladies present with record of high blood pressure, visual disturbances, proteinuria, generalized body swelling and seizures. Symptoms of pre-eclampsia
Pre-eclampsia can occur due to recurrent thrombosis of the placental bed. Pregnant ladies present with record of high blood pressure, visual disturbances, proteinuria, generalized body swelling and seizures.
Neurological complications Seizures and chorea can be the presenting complains of Anti- Phospholipid Syndrome (APS). Patients with APS can also develop transverse myelitis, causing sensory and motor impairment below the affected spinal cord level. Neurological complications
Seizures and chorea can be the presenting complains of Anti- Phospholipid Syndrome (APS). Patients with APS can also develop transverse myelitis, causing sensory and motor impairment below the affected spinal cord level.
Cutaneous manifestations Patients with APS can have skin ulcerations and digital gangrenes. Cutaneous manifestations
Patients with APS can have skin ulcerations and digital gangrenes.
Symptoms of venous thrombosis Deep vein thrombosis presents with severe pain and swelling of the extremities commonly affecting the left lower limb. This may be due to the compression of the left common iliac vein by the enlarged uterus.
Sudden severe chest pain, cough and hemoptysis are classic symptoms of pulmonary embolization.
Symptoms of venous thrombosis
Deep vein thrombosis presents with severe pain and swelling of the extremities commonly affecting the left lower limb. This may be due to the compression of the left common iliac vein by the enlarged uterus.
Sudden severe chest pain, cough and hemoptysis are classic symptoms of pulmonary embolization.
Symptoms of recurrent arterial thrombosis Patients with APS are at risk of thrombotic complications. They can present with sudden neurological impairment or with focal weakness due to cerebrovascular accidents.
Development of ischemic limbs and distal gangrenes is another presentation.
Sudden severe abdominal pain can occur due to mesenteric angina secondary to mesenteric thrombosis.
Symptoms of recurrent arterial thrombosis
Patients with APS are at risk of thrombotic complications. They can present with sudden neurological impairment or with focal weakness due to cerebrovascular accidents.
Development of ischemic limbs and distal gangrenes is another presentation.
Sudden severe abdominal pain can occur due to mesenteric angina secondary to mesenteric thrombosis.
Multiorgan failure Patients with catastrophic antiphospholipid syndrome (CAPS), a severe form of the disease can present with multiorgan failure. It is due to multiple thrombotic phenomena affecting major organs' perfusion. This can occur during the pregnancy and also during the postpartum period. Multiorgan failure
Patients with catastrophic antiphospholipid syndrome (CAPS), a severe form of the disease can present with multiorgan failure. It is due to multiple thrombotic phenomena affecting major organs' perfusion. This can occur during the pregnancy and also during the postpartum period.
History of recurrent miscarriages Patients with APS have a history of recurrent spontaneous miscarriages. These miscarriages usually occurs between 10th to 24th weeks of gestation. History of recurrent miscarriages
Patients with APS have a history of recurrent spontaneous miscarriages. These miscarriages usually occurs between 10th to 24th weeks of gestation.
History of autoimmune diseases Patients with other autoimmune diseases (like systemic lupus erythematosus) can manifest secondary APS (primary APS refers to the occurrence of APS with no other associated autoimmune diseases). History of autoimmune diseases
Patients with other autoimmune diseases (like systemic lupus erythematosus) can manifest secondary APS (primary APS refers to the occurrence of APS with no other associated autoimmune diseases).
Fetal and neonatal complications APS can cause preterm labour, oligohydramnios, intrauterine growth restriction and fetal distress. Fetal and neonatal complications
APS can cause preterm labour, oligohydramnios, intrauterine growth restriction and fetal distress.

Clinicals - Examination

Fact Explanation
Signs of cutaneous involvement Livedo reticularis, skin necrosis and ulceration and digital gangrenes can be seen in patients with APS. Some patients have photosensitive rashes. Signs of cutaneous involvement
Livedo reticularis, skin necrosis and ulceration and digital gangrenes can be seen in patients with APS. Some patients have photosensitive rashes.
Auscultation of the heart Cardiac auscultation can reveal mitral valve and aortic valve regurgitation. Mitral regurgitation produces a high pitched pansystolic murmur, whereas aortic regurgitation produces an early diastolic murmur. Auscultation of the heart
Cardiac auscultation can reveal mitral valve and aortic valve regurgitation. Mitral regurgitation produces a high pitched pansystolic murmur, whereas aortic regurgitation produces an early diastolic murmur.
Blood pressure Assessment of the blood pressure is important as maternal hypertension is a common association of APS. Blood pressure
Assessment of the blood pressure is important as maternal hypertension is a common association of APS.

Investigations - Diagnosis

Fact Explanation
Antiphospholipid antibody (aPL) aPL antibody is usually present in almost all patients with APS, but its mere presence does not diagnose APS. Antiphospholipid antibody (aPL)
aPL antibody is usually present in almost all patients with APS, but its mere presence does not diagnose APS.
Lupus anticoagulants (LA) LA is an autoantibody which prolong the clotting time by reducing the coagulant potential. Lupus anticoagulants (LA)
LA is an autoantibody which prolong the clotting time by reducing the coagulant potential.
Anticardiolipin antibodies (aCL) Similar to LA, aCL antibody reduces the coagulant potential and prolongs the activated partial thromboplastin time. Presence of LA or aCL antibodies and clinical symptoms of APS is necessary for making the diagnosis of APS. Anticardiolipin antibodies (aCL)
Similar to LA, aCL antibody reduces the coagulant potential and prolongs the activated partial thromboplastin time. Presence of LA or aCL antibodies and clinical symptoms of APS is necessary for making the diagnosis of APS.
Coagulation profile Activated partial thromboplastin time is usually prolonged due to the presence of LA and aCL antibodies. Assessment of dilute Russell Viper venom time (dRVVT), kaolin clotting time and plasma clotting time can also be done and all are prolonged. Coagulation profile
Activated partial thromboplastin time is usually prolonged due to the presence of LA and aCL antibodies. Assessment of dilute Russell Viper venom time (dRVVT), kaolin clotting time and plasma clotting time can also be done and all are prolonged.
Full blood count Thrombocytopenia, and anemia can be detected in full blood count. Full blood count
Thrombocytopenia, and anemia can be detected in full blood count.

Investigations - Management

Fact Explanation
Full blood count Low platelet levels can be observed in HELLP syndrome. Full blood count
Low platelet levels can be observed in HELLP syndrome.
Blood picture Patients with APS can develop HELLP syndrome and hemolytic anemia. Blood picture
Patients with APS can develop HELLP syndrome and hemolytic anemia.
Hepatic transaminases Elevated transaminases are a component of HELLP syndrome. Hepatic transaminases
Elevated transaminases are a component of HELLP syndrome.
Renal function test Patients with APS can develop renal infarction and cortical necorsis leading to deminished renal function. Altered electrolyte profile and raised serum creatinine are indicative of renal failure. Renal function test
Patients with APS can develop renal infarction and cortical necorsis leading to deminished renal function. Altered electrolyte profile and raised serum creatinine are indicative of renal failure.
Fetal ultrasound scan Multiple placental infarctions can cause placental insufficiency and intrauterine growth retardation. Measurement of amniotic fluid index is also important as oligohydroamnios is another recognized complication of APS. If intrauterine growth retardation is suspected fetal ultrasound scan should be repeated every 3-4 weeks. Uterine and umbilical artery Doppler assessments are indicated to assess the fetal well being and perfusion. Fetal ultrasound scan
Multiple placental infarctions can cause placental insufficiency and intrauterine growth retardation. Measurement of amniotic fluid index is also important as oligohydroamnios is another recognized complication of APS. If intrauterine growth retardation is suspected fetal ultrasound scan should be repeated every 3-4 weeks. Uterine and umbilical artery Doppler assessments are indicated to assess the fetal well being and perfusion.
Antiphospholipid antibody (aPL) Although universal screening for APS is not indicated, if the patient has symptoms and signs suggestive of APS (recurrent miscarriages, thrombotic phenomena) assessment of aPL is indicated. Antiphospholipid antibody (aPL)
Although universal screening for APS is not indicated, if the patient has symptoms and signs suggestive of APS (recurrent miscarriages, thrombotic phenomena) assessment of aPL is indicated.

Management - Supportive

Fact Explanation
Health education Patients with APS and pulmonary hypertension should be advised not to get pregnant. They should be using a permanent method of contraception.
It is better to delay the pregnancy, if the patient has uncontrolled hypertension or recent thrombotic events.
All patients should consult proper medical advise before getting pregnant and should be followed up closely during the period of pregnancy.
Patients should be educated about the possible maternal and fetal complications. They should be capable of identifying the complications of the disease (thrombotic phenomena, seizures, impending eclampsia).
Health education
Patients with APS and pulmonary hypertension should be advised not to get pregnant. They should be using a permanent method of contraception.
It is better to delay the pregnancy, if the patient has uncontrolled hypertension or recent thrombotic events.
All patients should consult proper medical advise before getting pregnant and should be followed up closely during the period of pregnancy.
Patients should be educated about the possible maternal and fetal complications. They should be capable of identifying the complications of the disease (thrombotic phenomena, seizures, impending eclampsia).

Management - Specific

Fact Explanation
High dose prednisone and low-dose aspirin Combination of high dose prednisone (40–60 mg daily) and low-dose aspirin (75 mg daily) is indicated in the treatment of APS during the early pregnancy. High dose prednisone and low-dose aspirin
Combination of high dose prednisone (40–60 mg daily) and low-dose aspirin (75 mg daily) is indicated in the treatment of APS during the early pregnancy.
Heparin and low-dose aspirin Heparin and low dose aspirin are used in the treatment of APS, as this reduces the incidence of thrombotic complications associated with APS.
Prophylactic heparin is indicated for patients with a prior history of recurrent miscarriages. If the patient has had a thrombotic complication (Eg: stroke) they need therapeutic doses of heparin to achieve full anticoagulation.
Low molecular heparin should be stopped at least before 12 hours of labor or planned Cesarean section and should be restarted during the post partum period to minimize the risk of deep vein thrombosis.
Heparin and low-dose aspirin
Heparin and low dose aspirin are used in the treatment of APS, as this reduces the incidence of thrombotic complications associated with APS.
Prophylactic heparin is indicated for patients with a prior history of recurrent miscarriages. If the patient has had a thrombotic complication (Eg: stroke) they need therapeutic doses of heparin to achieve full anticoagulation.
Low molecular heparin should be stopped at least before 12 hours of labor or planned Cesarean section and should be restarted during the post partum period to minimize the risk of deep vein thrombosis.
Intravenous immunoglobulin Intravenous immunoglobulin when used in high doses is proven to reduce the pregnancy related complications. Intravenous immunoglobulin
Intravenous immunoglobulin when used in high doses is proven to reduce the pregnancy related complications.

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