Background and aims Neonatal lupus erythematosus (NLE) is a passively acquired autoimmune disease of infancy, caused by the transplacental passage of maternal autoantibodies, mostly anti-RO/SSA and anti-LA/SSB. NLE presents with a transient rash and/or congenital heart block (CHB). The risk of developing NLE in SSA-positive women is ˜2%, however the risk increases to 25%, if the mother has had a previous child with NLE.
Objectives We present a case of NLE characterised by a third-degree CHB, ascites and life-threatening pericardial effusion, which was treated twice with intrauterine pericardiocentesis in week 22 and 29. After birth the child was treated with systemic corticosteroid on and off for 1 year, and she later received a pacemaker and was treated with ACE inhibitor and diuretics due to heart failure. Now, at the age of 6 years, she is still treated with ACE inhibitor. Her older sister also had NLE and her mother was found to have asymptomatic anti-SSA >100 U/ml.
Discussion This case is exceptional, as the fetus had severe exudative pericarditis and had life-saving pericardiocentesis performed in utero. We want to draw the clinicians’ attention to the increased risk of NLE, when a mother earlier has given birth to a child with NLE. Regular fetal echocardiography is important from week 16. In case of first- and second-degree CHB, maternal corticosteroid can be tried to reverse the condition. Also, treatment with hydroxychloroquine or IVIG may decrease the risk of CHB.
Statistics from Altmetric.com
If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.