Neonatal lupus is a passively transferred autoimmune disease that occurs in about 1-2 percent of babies born to mother with autoimmune disorders including SLE, Sjoegrens syndrome and to women with antibodies against Ro/SSA and La/SSB in the absence of autoimmune disease. Neonatal lupus are caused by passage of the anti-RO/SSA and/or anti-LA/SSB from mother to child after the 20 week of pregnancy
Cutaneous manifestations: 5%-10%. Recurrence: 15-25%. Manifestations: Usually it manifests in the first 2 weeks of life, [erythromatous anular lesions or arcuale macules] located primarily on the scalp and periorbital area] disappears spontaneously within 6 months but hypo-pigmentation and telangiecstasia may persist up to 2 years. Hematologic manifestations such as thrombocytopenia and congenital heart block may occasionally occur. Sunlight should be avoided. Most symptoms resolve within few months. CONGENITAL HEART BLOCK (CHB) IN one of 20,000
The most vulnerable period is from 16-24 weeks and it rarely develops after 30 weeks of pregnancy. The RO/LA antibodies causing fibrosis in the conductive system in the heart including the AV nodules. Among all causes of congenital complete heart block, neonatal lupus is responsible in 90-95% of cases. More than 90% of the mothers of affected offsprings have anti RO antibodies and 50-70% have anti LA antibodies (<1% in general population). CHB is found in 2% of mothers with antibodies. Diagnosis: Bradycardia and anti RO/LA with cardiac failure eventually causing hydrops (80% mortality). Congenital heart block in first trimester with bradycardia, nuchal translucency and severe cardiac anomalies have extremely poor prognosis. Management: In case of suspected myocarditis: steroids and plasmapheresis may be successfully used but are without effect on the conductive system. Some suggest Dexamethasone (cross placenta) from 24 weeks through the end of pregnancy. Incomplete heart block has been reversible on steroid therapy and has suppressed the associated pleuropericardial effusion and hydrops as well as antibodies, but the stsudy is based on limited data. Half of the patients who survived need pacemaker in later life. Neonatal mortality is high (20%). Often delivered by Cesarean section because of difficulties to interpret CTG and achieve optimal neonatal service. Pulse oximetry can be used during labour. Cases of incomplete heart bloch has been reversed when dexamethason and betamethason has been given in the rest of pregnacy. References: (1) Nelson-Piercy C. Handbook of Obstetric Medicine published in the United Kingdom in 2002 by Martin Dunitz Ltd. (2)www.uptodate.com 2007 UpToDate 2005, Online 13.3
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