Lupus in Pregnancy

Background

• The effect of pregnancy on systemic lupus erythematosus (SLE) disease activity is variable.
  • Patients who are clinically stable at conception tend to remain in remission.
  • There is a significant risk of flares if there was lupus disease activity in the 6 months prior to conception.
  • Pregnancy-related flares may occur at any time during pregnancy and for several months after delivery.
• Pregnancy with SLE increases maternal and fetal risks including pregnancy loss, preterm birth, fetal growth restriction, neonatal lupus, hypertension, and progression of renal disease.
• Neonatal lupus, which may result in congenital heart block, is associated with exposure to maternal anti-SSA/Ro or anti-SSB/La antibodies and occurs in 3.5%-8% of pregnancies in women with SLE.

Evaluation

• Initial laboratory tests (in addition to standard evaluation for pregnant women) should include complement levels, anti-double-stranded DNA (dsDNA) antibodies, anticardiolipin antibodies, lupus anticoagulant, anti-SSA and anti-SSB (anti-Ro and anti-La) and 24-hour urine studies.
• Monitor disease activity and effects on pregnancy with more frequent prenatal visits and fetal assessments.
  • Starting at 20 weeks schedule visits every 2 weeks (from standard 4-week interval) paying particular attention to signs of preeclampsia which occurs after 20 weeks.
  • Starting at 28 weeks begin weekly visits.

Management

• If possible, delay conception until systemic lupus erythematosus (SLE) has been in remission for at least 6 months.
• For SLE flares during pregnancy:
  • consider acetaminophen for arthralgia and hydroxychloroquine for arthritis and skin manifestations
  • use corticosteroids for lupus flares not adequately controlled by acetaminophen or hydroxychloroquine
• Patients with lupus nephritis:
  • If active disease or receiving treatment with potentially teratogenic effects, counsel patients to avoid pregnancy for ≥ 6 months after disease becomes inactive.
  • Continue hydroxychloroquine during pregnancy and initiate low-dose aspirin before 16 weeks gestation to decrease risk of pregnancy complications.
  • Corticosteroids, hydroxychloroquine, azathioprine, and calcineurin inhibitors are the only immunosuppressants considered safe in pregnancy.
• Treat hypertension whether due to chronic hypertension, lupus nephritis, or preeclampsia.
• When antimalarials (such as hydroxychloroquine) are indicated, continue them during pregnancy and lactation.
• Patients with obstetric antiphospholipid antibody syndrome (APS):
  • In patients with a history of recurrent pregnancy loss, administer antenatal heparin plus low-dose aspirin throughout pregnancy (Strong recommendation), with treatment beginning as soon as pregnancy is confirmed.
  • In patients with history of delivery < 34 weeks gestation as a result of eclampsia or severe preeclampsia due to placental insufficiency, consider low-dose aspirin or low-dose aspirin plus prophylactic dose heparin after taking into consideration patient risk profile (Weak recommendation).
  • In patients with history of preeclampsia or fetal growth restriction, low-dose aspirin is recommended.
  • In patients with obstetric APS with recurrent pregnancy complications despite low-dose aspirin and prophylactic heparin therapy:
    • consider 1 of (Weak recommendation):
      • increasing heparin dose to therapeutic dose
      • addition of hydroxychloroquine or low-dose prednisolone in first trimester
      • IV immunoglobulins (IVIG) in highly selected cases
    • addition of pravastatin is another option
  • In patients who have had a thrombotic event, consider low-dose aspirin plus therapeutic dose heparin during pregnancy (Weak recommendation).
  • Consider supplementary fetal surveillance with Doppler ultrasound and biometric parameters especially in third trimester to screen for placental insufficiency and small for gestational age fetuses (Weak recommendation).
• After cesarean delivery, prophylactic low-molecular-weight heparin (or mechanical prophylaxis if contraindicated) is suggested until discharge from the hospital (Weak recommendation).