P09 Neonatal cutaneous lupus: like mother like son

Abstract

Background: Neonatal lupus (nSLE) affects 1-5% of pregnancies of Ro-positive women and is the most common cause of isolated congenital complete heart block. We present the case of a boy with cutaneous nSLE whose mother was known to have Ro-positive Sjo gren's syndrome (SS). Method(s): The baby boy was born at 38 weeks, with initial APGAR score 9 and weight 3.155kg. Obstetric ultrasound and CTG monitoring had all been unremarkable. At two hours he developed poor respiratory effort and required ventilator support for 24 hours. ECG at this time was normal. He was noted to have two bruise like lesions on his forehead. By ten weeks, he had further annular erythematous lesions on his forehead and scrotum. His bloods showed the presence of circulating Ro antibodies. His full blood count and liver function tests were normal. His mother was diagnosed with SS on the basis of positive Ro antibodies, positive lip biopsy, neutropenia, lymphopenia and reduced complement. She presented with livedo reticularis, SICCA symptoms and Raynauds. She was unable to tolerate hydroxychloroquine. Prenatal testing revealed positive cardiolipin immunoglobulin and prophylactic heparin and aspirin were taken throughout pregnancy. Result(s): nSLE is a well recognised but rare complication in Ropositive mothers, whom are often asymptomatic themselves. A rash consistent with subacute cutaneous lupus is the most common presentation. This rash may be present at birth or develop within the first few weeks. Management includes sun protection advice and topical steroids for more serious cases. Most rashes resolve spontaneously as maternal antibodies clear after 17 weeks. Occasionally infants have residual telangiectasia or dyspigmentation. Cardiac conduction defects including complete heart block are the most severe symptom. Maternal fluorinated steroids such as dexamethasone may help reduce the risk of progression to complete heart block. Regular cardiac monitoring from 18 weeks is important. Haematological, hepatobiliary, central nervous and pulmonary involvement have also been described with multisystemic symptoms possible. nSLE is usually associated with transmission of Ro antibodies but can also be seen with La and U1-RNP antibodies. Ro52 and La48 are associated with cardiac complications whilst Ro50 and U1RNP are associated with skin manifestations. Other maternal and foetal risk factors of nSLE are unclear. nSLE recurs in up to 50% of further pregnancies. Cardiac conduction defects can occur even if not previously a clinical feature. It is unclear whether children with previous nSLE have an increased risk of developing autoimmune conditions in later life over and above the rate conferred by family history of autoimmunity. Conclusion(s): We present a case of cutaneous nSLE and discuss the current literature regarding this. There are several areas for future research including prevention of progression to complete heart block, antenatal risk stratification and long term autoimmune outcomes for children.