Systemic lupus erythematosus in Pregnancy

Systemic lupus erythematosus in Pregnancy

• SLE is known to increase the risk of spontaneous miscarriage; it can also cause fetal growth restriction and increased rates of sudden intrauterine death, pre-eclampsia and preterm delivery

Pre-pregnancy counselling :

• The presence of anti-Ro/La and 
  antiphospholipid antibodies should  be determined. 
  These antibodies are associated with congenital heart block 
  and neonatal cutaneous lupus syndrome. 
  Antiphospholipid 
  antibodies are present in about 30% of women with SLE and 
  are associated with arterial and venous thrombosis, recurrent 
  miscarriage, fetal growth restriction, fetal loss and preterm 
  delivery due to uteroplacental insufficiency.
• Quiescent disease, without associated antiphospholipid syndrome (APS), hypertension or renal involvement the risks of miscarriage/stillbirth and fetal growth restriction are not significantly increased
• The risk of miscarriage and stillbirth in pregnancies complicated by lupus varies in the literature from 6–35% and from 0–22%
• The great concern are women with SLE and associated pulmonary arterial hypertension who wish to conceive: maternal mortality rates have been quoted as being as high as 33%
• Women should  be advised not to conceive during a period of active disease, particularly with lupus nephritis, because of worse maternal and fetal outcomes
• Counsel women with SLE for increased risk of pre-eclampsia, preterm delivery and fetal growth restriction
• In active lupus nephritis with worsening renal function, increasing proteinuria and hypertension,it may be necessary to use such treatments as cyclophosphamide and mycophenolate mofetil, which are associated with congenital malformations if used in the first trimester,
• Pregnant women with active SLE/lupus nephritis or anti-Ro/La/antiphospholipidantibodies should be considered as a higher risk group and managed in tertiary centre
• If severe disease, patients need hospitalisation
• For patients with stable disease, 4-weekly reviews of disease activity and regular assessment of fetal growth, blood pressure and proteinuria are acceptable.
• For those women who are anti-Ro/La positive the fetal heart rate should be monitored and recorded at each visit and fetal echocardiography assessments made at 18–20 and ~28 weeks of gestation
• Women who have had a previous venous thromboembolism  should receive thromboprophylaxis with low molecular weight heparin throughout pregnancy and for 6 weeks postpartum 
• The risk of an SLE flare in pregnancy is increased with active disease in the 3–6 months prior to conception, with the majority of flares occurring after the 20th weeks of pregnancy
• Most flares are  managed expectantly with medical management and adjustments to drug therapy
• At every antenatal visit, measure the BP, test the urine for proteinuria,  and quantify the proteinuria by PCR or 24 hour urine proetien estimation.
• The features of lupus nephritis include hypertension and proteinuria with or without haematuria and renal impairment
• The presence of hematuria or red cell casts as well as a rise in anti-dsDNA titres or a fall in complement levels help to distinguish this from pre-eclampsia
• In cases of lupus nephritis that fail to respond to increasing dosages of steroids and azathioprine, and where there is a deterioration of renal function and/or hypertension, other immunosuppressive drugs may be considered, such as mycophenolate mofetil or tacrolimus. Such management decisions should be undertaken in consultation with nephrologists and rheumatologists.
• Premature rupture of membranes is also regarded as being more frequent in pregnancies complicated by SLE; rates vary and are generally quoted as ~20% and is common in women who are on steroids

SLE and fetal risks

• Increased rates of miscarriage,
• Increased risks of congenital heart block, fetal growth restriction and increased rates of preterm delivery 
• Scanning at least every 4 weeks to screen for fetal growth restriction in those women at risk is generally accepted and fetal echocardiography referral should be arranged for those with anti-Ro/La antibodies
• Doppler studies can be used to estimate placental function
• A uterine artery Doppler should be first carried out at 20 weeks and repeated 4 weeks later if any abnormality is found
• A raised pulsatility index or diastolic notching are associated with increased risk for developing  pre-eclampsia, as they can indicate underlying placental dysfunction
• Congenital heart block is associated with maternal anti-Ro/La autoantibodies.
• usual presentation is a fixed fetal bradycardia of 60–80 beats per minute on ultrasound scan.
•  It occurs in 2–3% of fetuses of women with the anti-Ro/La antibody 
• CHB is associated with significant perinatal morbidity and mortality, with about half of infants requiring pacing by the first year of life. 
• Congenital heart block develops between 18– 28 weeks of gestation and fetal echocardiography should be performed around this period to detect it. Hydrops fetalis can occur in utero and is thought to be due to the degree of endomyocardial fibrosis and associated myocarditis.

Neonatal lupus rash

• lesions are  commonly seen on the face and scalp 
  and appear typically after ultraviolet exposure in the first 
  2 weeks of life, but can appear  up to 3–6 months postpartum

Drug treatment in SLE

• Glucocorticoids, mainly in the form of prednisolone, are frequently but not exclusively used as one of the first-line treatments in pregnancy
• Women on moderate to high dosages of steroids should should be screened regularly for gestational diabetes.
• Immunosuppressants  that are used and are generally considered safe during pregnancy include azathioprine and hydroxychloroquine. There is no  need to discontinue them during pregnancy

Postpartum management

• To consider thromboprophylaxis
• Manage hypertension

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