Morbidity and mortality of neonatal lupus

  Neonatal lupus is a major concern for lupus patients who are pregnant. It is a passively transmitted autoimmune disease with a prevalence of approximately 5% in the children of mothers with SLE. Once a child with neonatal lupus is born, the chance of that mother developing the disease in her next child increases to 15 percent.  Studies have also shown that most infants with neonatal lupus have mothers who are positive for SSA and SSB antibodies and positive for HLA A1, Cw7, B8, DR3. The incidence of HLA DQA1*03 and DQB1*03 was significantly higher in affected infants, and the incidence of children with neonatal lupus was higher in mothers with increased titers of SSA and SSB antibodies.  Most fetuses with neonatal lupus present with a mild syndrome consisting of transient erythema, mainly on the scalp and periorbital areas, usually following UV exposure in the delivery room. Because the half-life of IgG antibodies is approximately 21-25 days, the syndrome is self-limiting and usually resolves within 3-6 months.  However, infants rarely develop symptoms as severe as congenital heart block. Congenital heart block can be diagnosed in the 18th-24th weeks of gestation when fetal bradycardia is detected. The fetus usually tolerates this condition well in the uterine environment, however, heart block is usually irreversible and there is a high incidence of stillbirth in the prenatal period. One review found that nearly 20% of affected children died early in life. Most survivors require a permanent pacemaker.  In addition to rash and congenital heart block, neonatal lupus has other manifestations: other cardiac abnormalities include right bundle branch block, second degree AV block, patent foramen ovale, aortic stenosis, tetralogy of Fallot, atrial septal defect, ventricular septal defect, mitral and tricuspid regurgitation, pericarditis and myocarditis.  Based on the potential cardiac manifestations of neonatal lupus and its associated morbidity and mortality, the following are recommended for women with SLE who are pregnant: (1), SSA antibody testing should be performed as early as possible in pregnancy, and if complete heart block occurs in the fetus, treatment with corticosteroids (betamethasone or dexamethasone) must be started in the 23rd week of pregnancy before birth and continued until the end of pregnancy. Although hormone therapy does not modify the heart block, hormones help to suppress the associated cardiac or pericardial effusion or myocarditis and improve the prognosis.  (2), The development of heart block is followed by fetal electrocardiography and associated cardiac abnormalities are identified.  (3), After delivery, the pediatrician should be prepared to implant a pacemaker.  (4), If the mother is SSA antibody positive and the fetus does not develop bradycardia throughout most of the pregnancy, the likelihood of heart block is minimal and, moreover, the cutaneous symptoms of neonatal lupus are benign and transient, thus there is no cause for concern.