Neonatal lupus is an autoimmune disease that is acquired in utero from antibodies produced by the maternal immune system against Ro and La antigens. It is characterized by varying degrees of heart block and can be associated with an erythematosus rash. Neonatal lupus occurs in 1–2% of babies born to mothers with anti-Ro (also known as anti-SSA) and anti-La (also known as anti-SSB) antibodies. It is not necessary for these women to have clinical manifestations of an autoimmune disease. The most notorious complications of neonatal lupus involve the heart. These include first-, second-, and third-degree heart block as well as endocardial fibroelastosis (EFE). Neonatal lupus is the etiology most commonly associated with isolated congenital heart block, accounting for up to 95% of all cases. This chapter will review the pathophysiology and current guidelines regarding management of pregnancies complicated by neonatal lupus.
Miliaresis C, Phoon C, Buyon J, Friedman D. Neonatal lupus. In: Nussinovitch U, editor. The heart in rheumatologic, autoimmune and inflammatory diseases: pathophysiology, clinical aspects and therapeutic approaches. London, UK: Elsevier; 2017. pp. 269-80.