Overview of Neonatal Lupus

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


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

In This Article

Long-term Outcomes

The majority of babies go on to live normal productive lives (Lee, 2009). Much remains to be studied regarding the long-term outcomes in NL (Inzinger et al., 2012). Cutaneous lesions along with other clinical findings, except third-degree heart block, will resolve with the dissipation of maternal antibodies out of the baby's body (Zuppa et al., 2008) up to 1 year (Monari, Gualdi, Fantini, & Giannetti, 2007). Babies with NL are at risk of having an autoimmune disease develop in their lifetime (Lee, 2009). Pain and Beresford (2007) noted that the risk of developing cardiomyopathy is 5% to 11% even if the baby receives a pacemaker, whereas Shahian and colleagues (2011) noted that morbidity increases up to 50% to 70%. Outcomes can vary among patients. Poor outcomes have been seen in neonates characterized as premature, low birth weight, having a low ventricular rate, and having significant structural heart disease. Evidence of ventricular dysfunction, cardiomyopathy, or hydrops fetalis also plays a role in adverse outcomes (Tunaoglu et al., 2010). Because third-degree block is irreversible (Lee, 2009), death may result from failure of an acquired pacemaker later in childhood (Blickstein & Friedman, 2011).

Telangiectic and slightly atrophic lesions can remain over the periorbital area, along with hyperpigmented macules (Laurinaviciene et al., 2012) and have been treated with laser therapy (Wisuthsarewong et al., 2011). Cutaneous lesions are considered benign in patients with NL but are important in the assessment of risk factors that may indicate a more serious disease in the mother and future siblings (Izmirly, Llanos, et al., 2010). Liver failure is significant for a poor prognosis; however, elevations in liver enzymes are transient, and elevation in direct bilirubin will resolve over time (Pain & Beresford, 2007).

In terms of the mother, women with a history of autoimmune disease should be evaluated for the presence of autoantibodies (Pain & Beresford, 2007). The recurrence rate of NL is 25% in subsequent pregnancies (Pain & Beresford, 2007). If the mother has anti–SS-A/Ro or anti–SS-B/La antibodies, recurrence of CHB in subsequent pregnancies is approximately 15% (Escobar, Gomez-Puerta, Albert, Ferrer, & Girona, 2007). Diagnosis of NL in a baby does not predict that the mother will progress to disease (Rivera et al., 2009); however, it is important to note that although the autoantibodies dissipate in the fetus or newborn, the mother would continue to possess these findings (Capone, Buyon, Friedman, & Frishman, 2012). Mothers at risk for autoimmune disease must be identified early to allow for evaluation, monitoring, and treatment sooner than later because the leading causes of death for these women are infection and renal failure (Nodine et al., 2011).

Among births with multiple neonates, studies have documented different presentations of NL between siblings (Cimaz et al., 2006). The authors studied CHB in births with multiple neonates and found that in a family of twins and a family of triplets, CHB developed in only one baby in each group of multiple neonates. In 2008, Terzic, Vucinic, Stimec, Dokic, & Kostic presented a case of bichorionic twins diagnosed with NL. Both male infants presented with a discoid head and facial skin lesions. Twin A was diagnosed with grade I-II intraventricular hemorrhage and was determined to be positive for ANA and RNP antibodies, with anti-Ro greater than 200 IU/ml. Twin B died from respiratory distress from unknown reasons, whereas Twin A made a full recovery. Sawalha and colleagues (2006) determined that 20% to 30% of identical twins are concordant for lupus. Because of the risk of developing an autoimmune disorder later in childhood (Hoath & Narendran, 2011) babies who survive NL should undergo long-term follow-up.