Neonatal lupus erythematosus – prevention is better than cure

Abstract

Neonatal lupus erythematosus (NLE) is a congenital autoimmune condition in which the transplacental passage of immunoglobulin G (IgG) directed against auto-antigens causes clinical symptoms in the foetus or neonate. Anti-Ro/ SS-A, anti-La/SS-B, and to a lesser extent, anti-U1RNP autoantibodies (aAbs) have the strongest association with NLE. However, ~ 50% of affected mothers are asymptomatic despite carrying those aAbs. The clinical picture of the disease is very diverse. Cardiac manifestations are the most severe, including congenital heart block (CHB), with a mortality rate of ~18%. Preventative therapy with hydroxychloroquine (HCQ) reduces the recurrence rate of CHB in subsequent pregnancies by ~50%.

A Comprehensive Literature Review on Managing Systemic Lupus Erythematosus: Addressing Cardiovascular Disease Risk in Females and Its Autoimmune Disease Associations

“…In addition to a high SLEDAI score, having lupus nephritis within six months before pregnancy is associated with higher rates of maternal complications including disease flares during pregnancy [ 142 , 143 ]. Other factors associated with adverse outcomes are major organ involvement, the presence of anti-Ro/La antibodies [ 144 , 145 ], and the presence of hypercoagulability [ 146 ]. Discontinuation of hydroxychloroquine is associated with the risk of disease flares during pregnancy and the postpartum period [ 147 , 148 ] as well as preeclampsia [ 149 ].…”