Issues regarding pregnancy in women with systemic lupus erythematosus (SLE) and Sjogren's Syndrome (SS) are an important part of disease management. SLE impacts women during their peak reproductive years while SS occurs more frequently at the tail end of this period.
Maternal risks Better disease control of SLE in general has improved the course of SLE during pregnancy. Nonetheless, pregnancy SLE flare rate still approaches 60 percent. Fortunately, only one-fifth of these flares will be severe. Common disease manifestations during pregnancy include lupus nephritis, cutaneous disease, arthritis and thrombocytopenia. The type of organ-system involvement in the pre-conception period may predict disease symptoms during pregnancy. Risks for flare during pregnancy include active disease in the six months preceding conception and any history of renal disease. Low complement levels and hematologic abnormalities also portend worse pregnancy outcome. Preeclampsia, eclampsia and HELLP syndrome are increased in lupus pregnancies, with 25 percent of women with SLE developing preeclampsia. Preeclampsia can be difficult to distinguish from SLE flare as both conditions are associated with hypertension, edema, proteinuria and low platelet counts. Elevated liver function tests and uric acid levels, an acellular urine sediment and onset after 34 weeks suggest preeclampsia, whereas low complement levels, rising anti-dsDNA antibody titers, low white blood cell count, an active urine sediment and onset prior to 20 weeks gestation are more indicative of a lupus flare. Higher rates of thrombosis, cesarean section and maternal mortality occur in women with SLE when compared to controls. SLE patients with pulmonary hypertension, prior cerebral vascular event or myocardial infarction are at the highest risk for maternal mortality. Limited data precludes conclusions regarding the maternal risks in SS pregnancy other than an apparent increase in cesarean section rate.
Fetal risks Increased rates of miscarriage and stillbirths are reported in SLE pregnancy with and without the presence of antiphospholipid antibodies (aPLs). Preterm premature rupture of the membranes (PPROM) with preterm birth also occurs with higher frequency in SLE pregnancies. Reports for the incidence of small for gestational age infants (SGA) in SLE varies from the background rate of 5% to an increased rate of 30%. There is conflicting data on whether SS causes increased fetal loss. In women with SLE and/or SS, the presence of anti-SS-A/anti-Ro and anti-SS-B /anti-La antibodies is associated with a 10-15% risk of neonatal lupus (cardiac, cutaneous, hematologic and hepatic manifestations) and a 2% risk of congenital complete heart block in the offspring. Risk of the latter increases to 17% in a subsequent pregnancy.
Management/Treatment Women with SLE and SS should be co-managed by a team that includes a maternal-fetal medicine and rheumatology specialist who are familiar with the management of these disorders during pregnancy. For women with SLE, baseline laboratory testing should be done prior to pregnancy or early in the first trimester. This evaluation should include a complete blood count, liver function tests, assessment of renal function including urine protein testing, uric acid level, anti-dsDNA titer, complement levels and testing for anti-SS-A/anti-Ro and anti-SS-B /anti-La and antiphospholipid antibodies. Patients with SS should also be assessed for the presence of anti-SS-A/anti-Ro and anti-SS-B /anti-La antibodies. Regular monitoring with laboratory testing should be tailored to the individual's clinical situation and antibody status. Treatment strategies should focus on disease control prior to conception. Certain medications commonly used for SLE disease management such as methotrexate, leflunomide, mycophenolate mofetil, cyclophosphamide, belimumab, and rituximab, are contraindicated during pregnancy. Other immunosuppressive agents such as cyclosporine, azathioprine, and tacrolimus are considered compatible with pregnancy. Glucocorticoids can be used during pregnancy although they carry an increased risk of cleft palate formation with first trimester exposure, and PPROM, gestational diabetes and hypertension when used throughout pregnancy. Transitioning women to medications that are compatible with pregnancy should occur six months prior to conception to ensure that patients are stable on their anticipated pregnancy regimen. Hydroxychloroquine use during pregnancy should be encouraged in SLE patients as the data suggest that this medication improves pregnancy outcome. Hydroxychloroquine should also be considered for all anti-SSA/anti-Ro and anti-SSB /anti-La positive mothers to reduce the risk of congenital complete heart block.
Disclosure of Interest None declared