Overview of Neonatal Lupus

Benay Johnson, MSN, RN, CPNP, NNP-BC


J Pediatr Health Care. 2014;28(4):331-341. 

In This Article


In Utero

A previous pregnancy resulting in a baby with NL is an indication that monitoring with serial fetal echocardiography should start at 16 weeks in patients with SLE (Lee, 2009). Prednisone is a glucocorticoid frequently used to manage SLE flares. Although data conflict, steroids are considered the primary medication to manage pregnancy in a patient with SLE (Smyth & Garovic, 2009). Prednisone and methylprednisolone cross the placenta in low concentrations (Bermas, 2011). A study by Zuppa and colleagues (2008) credits prenatal steroids for a good outcome in neonates. Administration of steroids may reverse first- and second-degree AV block but not third-degree or complete block (Friedman et al., 2008). Friedman and colleagues (2009) found that steroids as a single agent failed to prevent the conversion of second-degree AV block to third-degree AV block. Because of the correlation between the level of autoantibodies and the development of cardiac NL anomalies, current and future treatment goals would be to decrease the levels of the autoantibodies (Jaeggi et al., 2010).

The antimalarial drug hydroxychloroquine (HCQ) is used in persons with mild lupus and Sjögren syndrome (Sawalha et al., 2006). HCQ has been studied as a way to decrease the risk of CHB in fetuses of mothers with SLE. Izmirly et al. (2010) performed a case-controlled study to evaluate the pregnancy outcomes of women with SLE in regard to cardiac manifestations in fetuses. The sample was obtained from two studies that were in progress to evaluate PR interval and dexamethasone in patients with cardiac manifestations of NL, predictors of pregnancy outcomes, and biomarkers in APS and SLE. Women were selected who had been diagnosed with SLE prior to pregnancy with documented anti–SS-A/Ro or anti–SS-B/La antibodies at pregnancy. Out of 201 study participants, 50 babies were diagnosed with cardiac manifestations as a result of NL. Out of this sample, seven were exposed to HCQ. Using a control group of 151, 56 were exposed to HCQ. Total exposure to HCQ was 63 out of a sample size of 201. The authors concluded that exposure to prenatal HCQ in mothers with SLE may decrease the risk of cardiac manifestations of NL. This study suggests that infants of women with anti-Ro/La antibodies who are treated with HCQ have a decreased occurrence of CHB.

IVIG is used to decrease the ability of the antibodies to pass through the placenta. It is hypothesized that the fetus and infant treated with the combination of IVIG and prednisolone do better than those treated with corticosteroids alone (Trucco et al., 2011). Makino, Yonemoto, Itoh, & Takeda (2007) used a sample size of 24 pregnant women to evaluate the effects of plasmapheresis with and without prednisolone on the development of CHB in babies born to mothers with SLE and/or Sjögren syndrome. Thirteen women were not treated with prednisolone. Only 2 of these 13 women received plasmapheresis. Of the remaining 11 women, prednisolone was started before conception, and five of these women also underwent plasmapheresis. Six cases of CHB emerged from the 24 pregnancies; four of the cases were from women who did not receive either prednisolone or plasmapheresis. One woman received both prednisolone and plasmapheresis and one woman received prednisolone but did not receive plasmapheresis. The authors concluded that the majority of babies with CHB were born to mothers who did not receive either treatment or that CHB can occur even though plasmapheresis and corticosteroids are administered.


The majority of women with high anti–SS-A/Ro levels ultimately have normal pregnancy outcomes (Jaeggi et al., 2010). Except for heart block, other findings of NL, whether cutaneous, hepatobiliary, or hematologic, will dissipate after 6 months because this is the life of the IgG antibody (Zuppa et al., 2008). Infants with ongoing cardiac disease who are unable to compensate for a slow heart rate may require pacing (Wisuthsarewong et al., 2011). As stated earlier, cutaneous and cardiac findings can manifest after discharge from the newborn nursery or intensive care unit. The primary care provider may be the first to encounter the baby with any possible finding of NL. Taking a complete maternal history is important, coupled with an in-depth physical examination. Dermatologic findings may differ between the mother and newborn, with the rash of the newborn being more of an annular erythematous presentation; it also can be located in the diaper area (Silverman & Jaeggi, 2010). Neonatal lupus may warrant administration of prednisolone and/or IVIG depending on the severity of symptoms (Krakowski & Admani, 2012). Depending on the presenting clinical features, consultation with dermatology and cardiology will enable the practitioner to provide optimal care to this patient.