What are the management recommendations for a female patient with Systemic Lupus Erythematosus (SLE) who wants to become pregnant?

Management of SLE in Pregnancy

Women with SLE who desire pregnancy should ideally wait until their disease has been inactive or in stable remission for at least 6 months, then continue hydroxychloroquine throughout pregnancy, add low-dose aspirin by 16 weeks gestation, and maintain close multidisciplinary monitoring with rheumatology and high-risk obstetrics. 1

Preconception Counseling and Risk Stratification

All women with SLE contemplating pregnancy require comprehensive preconception counseling to assess risk factors and optimize disease control before conception. 2, 1

Major Risk Factors for Adverse Outcomes

The following factors significantly increase maternal and fetal complications:

• Active or flaring SLE at conception - particularly active lupus nephritis 2, 1
• History of lupus nephritis - even if currently inactive 2, 3
• Presence of antiphospholipid antibodies or antiphospholipid syndrome (APS) 2, 1
• High-risk antiphospholipid antibody profile - lupus anticoagulant, multiple antibodies, or moderate-to-high titers 2
• History of thrombotic events 2
• Positive anti-Ro/SSA or anti-La/SSB antibodies - risk for neonatal lupus and congenital heart block 2, 4

Optimal Timing for Conception

Pregnancy should be planned only when SLE has been inactive or in stable remission for 6-12 months. 1 Active disease at conception dramatically increases the risk of disease flares during pregnancy and adverse outcomes for both mother and fetus. 2, 5

Medication Management Before and During Pregnancy

Essential Medications to Continue

Hydroxychloroquine must be continued preconceptionally and throughout pregnancy for all SLE patients unless contraindicated. 1, 6 This is the single most important medication intervention, as it:

• Reduces disease activity and prevents flares 1, 3
• Improves obstetrical outcomes 1, 3
• Is safe throughout pregnancy and lactation 6
• May reduce the risk of congenital heart block in anti-Ro/SSA positive mothers 7

Low-dose aspirin (81 mg daily) should be started preconceptionally or no later than gestational week 16. 1, 8 This reduces the risk of preeclampsia, particularly in patients with lupus nephritis or antiphospholipid antibodies. 2, 1

Safe Immunosuppressive Options During Pregnancy

For patients requiring immunosuppression to maintain disease control:

• Oral glucocorticoids - use the lowest effective dose, ideally ≤7.5 mg/day prednisone equivalent 2, 1
• Azathioprine - safe throughout pregnancy for maintenance therapy 2, 1, 8
• Calcineurin inhibitors (tacrolimus, cyclosporine A) - safe options for disease control 2, 8

Medications That Must Be Discontinued

The following medications are teratogenic and must be stopped before conception:

• Mycophenolate mofetil/mycophenolic acid - discontinue at least 6 weeks before attempting conception 2, 8
• Methotrexate - teratogenic, must be stopped 2, 8
• Leflunomide - teratogenic, requires washout 2, 8
• Cyclophosphamide - avoid during pregnancy 2, 8
• ACE inhibitors and ARBs - discontinue immediately upon pregnancy confirmation due to first-trimester teratogenic effects; switch to nifedipine or labetalol 1

Special Management for Antiphospholipid Antibodies/Syndrome

Women with positive antiphospholipid antibodies or definite APS require combination anticoagulation therapy throughout pregnancy. 2, 1

The standard regimen is:

• Low-dose aspirin (81 mg daily) PLUS
• Low-molecular-weight heparin or unfractionated heparin throughout pregnancy 2, 1

This combination significantly decreases the risk of pregnancy loss and other adverse pregnancy outcomes. 2

Monitoring Protocol During Pregnancy

Maternal Disease Activity Monitoring

Pregnant women with SLE require regular assessment by a multidisciplinary team including rheumatology and high-risk obstetrics. 1, 4

Laboratory monitoring should include at least once per trimester (more frequently if disease is active): 4

• Complete blood count with differential 4
• Renal function parameters - blood urea nitrogen, creatinine 2, 4
• Urinalysis and urine protein-to-creatinine ratio 4
• Serological markers - serum C3/C4 complement levels and anti-dsDNA antibody titers 2, 4
• Blood pressure monitoring - essential at every visit 2

Autoantibody Testing

Anti-Ro/SSA and anti-La/SSB antibodies should be tested once before or early in pregnancy. 4 If positive, these antibodies require:

• Fetal echocardiography monitoring for congenital heart block, especially if fetal dysrhythmia or myocarditis is suspected 2, 7
• Serial echocardiograms typically starting at 16-18 weeks gestation through 26-28 weeks 7

Fetal Surveillance

All pregnant women with SLE and/or APS should undergo supplementary fetal monitoring, particularly in the third trimester: 2

• Doppler ultrasonography - to screen for placental insufficiency 2
• Biometric parameters - to detect intrauterine growth restriction and small-for-gestational-age fetuses 2

Management of Disease Flares During Pregnancy

Moderate-to-severe lupus flares during pregnancy can be managed with: 2

• Intravenous glucocorticoid pulse therapy 2
• Intravenous immunoglobulin 2
• Plasmapheresis in severe cases 2

The key is early recognition and prompt treatment to prevent maternal and fetal complications. 9

Postpartum Management

The postpartum period is high-risk for disease flares, particularly renal flares. 1 Close surveillance is essential during the first 3-6 months postpartum. 1

Breastfeeding Compatibility

The following medications are compatible with breastfeeding: 1, 6

• Hydroxychloroquine - present in breast milk at low levels, no adverse effects reported 6
• Prednisone - compatible with breastfeeding 1
• Azathioprine - compatible with breastfeeding 1

Common Pitfalls to Avoid

• Failing to achieve disease remission before conception - active disease at conception is the strongest predictor of poor outcomes 2, 1
• Discontinuing hydroxychloroquine during pregnancy - this medication should never be stopped unless absolutely contraindicated 1, 6
• Delaying aspirin initiation - must start by 16 weeks gestation for preeclampsia prevention 1
• Inadequate monitoring frequency - these pregnancies require more frequent visits than standard prenatal care 4
• Missing the diagnosis of congenital heart block - requires serial fetal echocardiography in anti-Ro/SSA positive mothers 2, 7
• Confusing preeclampsia with lupus nephritis flare - both can present with proteinuria and hypertension, requiring careful clinical distinction 7