Adverse perinatal outcome rates are signiicantly elevated in pregnancies complicated by SLE. Among these are preterm delivery, fetal-growth restriction, stillbirth, and neonatal lupus syndrome (Madazli, 2014; Phansenee, 2017). Perinatal outcome rates worsen in mothers with a lupus flare, significant proteinuria, or renal impairment and in those with chronic hypertension, preeclampsia, or both (Lazzaroni, 2016). Adverse outcomes are also more common in women with neuropsychiatric lupus (de Jesus, 2017). Reasons at least partially responsible for adverse fetal consequences include decidual vasculopathy with placental infarction and decreased perfusion (Hanly, 1988).
This is characterized by newborn skin lesions-lupus dermatitis; a variable number of hematological and systemic derangements; and occasionally congenital heart block (Hahn, 2015). Cutaneous manifestations can be present in 30 to 40 percent of infants and appears at 4 to 6 weeks of age (Silverman, 2010). hese are usually associated with anti-SS-A and SS-B antibodies, and approximately 40 percent of women with SLE are positive for these (Buyon, 2015). Thrombocytopenia and hepatic involvement are seen in 5 to 10 percent of afected infants.
In a review of outcomes in 91 infants born to women with lupus, eight of these were possibly afected-four had deinite neonatal lupus and four had possible disease (Lockshin, 1988). Cutaneous lupus, thrombocytopenia, and autoimmune hemolysis are transient and clear within a few months (Zuppa, 2017). This is not always so for congenital heart block, discussed next. In subsequent ofspring, the recurrence risk for neonatal lupus may near 25 percent Qulkunen, 1993).
Fetal and neonatal heart block results from difuse myocarditis and ibrosis in the region between the atrioventricular (A ) node and bundle of His. Congenital heart block develops almost exclusively in fetuses of women with antibodies to the SS-A or SS-B antigens (Buyon, 1993). Even in the presence of such antibodies, however, the incidence of fetal myocarditis is only 2 to 3 percent but rises to 20 percent with a prior afected child (Bramham, 2012; Lockshin, 1988). Fetal cardiac monitoring is performed between 18 and 26 weeks' gestation in pregnancies with either of these antibodies. The cardiac lesion is permanent, and a pacemaker is generally necessary. Long-term prognosis is poor. Of 325 infants with cardiac neonatal lupus, nearly 20 percent died, and of these, one third was stillborn (Izmirly, 2011).
Maternal administration of corticosteroids, plasma exchange, or intravenous immunoglobulin does not reduce the risk of congenital heart block. Maternal corticosteroid administration for treatment congenital heart block is controversial, is currently not recommended, and is discussed further in Chapter 16 (p. 316). Although this therapy to treat fetal heart block has not been studied in randomized trials, some evidence suggests that early fetal exposure to the mother's corticosteroid treatment for SLE may mitigate fetal myocarditis. Namely, Shinohara and coworkers (1999) reported no heart block in 26 neonates whose mothers received corticosteroids before 16 weeks' gestation as part of SLE maintenance therapy. By contrast, 15 of 61 neo nates with heart block were born to women in whom corticoste roid therapy was begun after 16 weeks for an SLE exacerbation.