Systemic Lupus Erythematosus (SLE) in Pregnancy

SUMMARY:  

SMFM has released guidance regarding SLE, an inflammatory autoimmune disorder involving multiple organs systems characterized by flares and remissions with associated immunologic abnormalities. It is most prevalent in young women and therefore often occurs during pregnancy. While antinuclear antibodies are associated with SLE, about 40% have anticardiolipin antibodies, and a smaller percentage have lupus anticoagulant. Complications of SLE include nephritis, hematologic, and neurologic abnormalities. During pregnancy, the diagnosis is often harder to identify due to the overlap in symptoms of normal pregnancies and is associated with an increase in morbidity. ACOG has endorsed these recommendations.

Prevalence

• Estimated to be 28 to 150 per 100,000 individuals
• SLE is several times more prevalent in females than male
• In the US, there are approximately 3300 deliveries per year in people with SLE

Diagnosing SLE

• There are different systems for diagnosing SLE (see ‘Learn More – Primary Sources’)
  • European Alliance of Associations for Rheumatology (EULAR)/American College of Rheumatology
  • Systemic Lupus International Collaborating Clinics (SLICC)
• In SLICC scoring model, diagnosis of SLE is possible with 2 criteria
  • Presence of biopsy-proven nephritis compatible with SLE and
  • Presence of ANA or anti-dsDNA antibodies
• In both systems, diagnosis also possible with scoring system that includes clinical and laboratory findings

Clinical  

• Acute cutaneous lupus
• Chronic cutaneous lupus
• Nonscarring alopecia
• Oral or nasal ulcers
• Synovitis involving ≥2 joints
• Serositis
• Neurologic: seizures, psychosis, stroke

Laboratory

• Renal: proteinuria >500 mg/24 h or red cell casts
• Hemolytic anemia
• Leukopenia or lymphopenia
• Thrombocytopenia

Immunologic testing

• ANA
• Anti-dsDNA
• Anti-Sm
• Antiphospholipid antibodies
• Low complement
• Direct Coombs test in the absence of hemolytic anemia

Diagnostic criteria for Antiphospholipid Syndrome (APS)

• Clinical criteria
  • Vascular thromboses with 1 or more episodes of arterial, venous, or small vessel events
  • Obstetrical
    • ≥1 unexplained death at ≥10 weeks in a structurally normal fetus
    • ≥1 preterm birth at <34 weeks due to severe preeclampsia or uteroplacental insufficiency
    • ≥3 unexplained losses at < 10 weeks gestation
• Laboratory criteria
  • Lupus anticoagulant on ≥2 occasions ≥12 weeks apart
  • Anticardiolipin antibodies IgG and/or IgM at medium or high titers on ≥2 occasions at least 12 weeks apart
  • Anti beta 2 glycoprotein antibody IgG and/or IgM > 99% on two occasions at least 12 weeks apart

Treatment

• To decrease the risk of preeclampsia, low dose aspirin should be recommended daily beginning at 12 weeks gestation until delivery.
  • Initial dose is 200 mg twice daily
• Avoid the following during pregnancy
  • > 48-hour use of nonsteroidal anti-inflammatory medications should be avoided
  • COX-2 inhibitors and full dose aspirin

KEY POINTS:

• Pregnancy planning
  • Discontinue methotrexate 1 to 3 months prior to conception
  • Discontinue mycophenolate mofetil/mycophenolic acid at least 6 weeks before conception
  • Disease should be in remission for six months prior to attempting conception
• If APS without a prior thrombotic event, prophylactic unfractionated or low molecular weight heparin with low dose aspirin is recommended
• Fetal heart block with SSA and SSB antibodies
  • Steroids should not be used to manage fetal heart block during pregnancy
  • Serial echocardiograms to check P-R interval are not recommended
• Fetal testing
  • No evidence to determine an optimal approach but SMFM recommends
    • Weekly surveillance by 32 weeks gestation
    • Growth scans monthly or at 28 and then at 32 to 34 weeks gestation
  • If complex/severe SLE (lupus nephritis, lupus flare, prior loss, antiSSA or SSB antibodies, or thrombosis)
  • Testing should be individualized in consultation with MFM
• Delivery management
  • If complex SLE or secondary complications such as preeclampsia, delivery should be individualized.
  • If uncomplicated SLE, delivery at 39 weeks is reasonable.
  • If prolonged steroids required during pregnancy
    • Stress dose steroids should be given for cesarean delivery
    • Individualized for vaginal delivery
• If severe maternal risk, such as lupus nephritis, pulmonary hypertension, recent stroke, or active renal, neurologic, cardiac, or pulmonary disease, pregnancy should be discouraged
• Contraception should be reviewed and discussed
  • Most patients can use long-acting reversible methods
  • Barrier methods are safe but are the least effective
    • Implants with etonogestrel should not be used in those using long term corticosteroids or APS due to uncertain risks to bone density and of thrombosis
    • Estrogen containing oral contraceptives contraindicated in those with APS or prior history of thrombosis.
    • Mycophenolate, cyclosporin and warfarin may decrease effectiveness of oral contraceptives
    • Theoretical risk to bone density from long term use of  Intramuscular and implantable progestins (e.g., DMPA)

Primary Care – Learn More

SMFM: Systemic lupus erythematosus in pregnancy

Calculator: SLICC Criteria for Systemic Lupus Erythematosus (SLE)