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Background Several studies have evaluated mortality and shortterm morbidity in neonatal lupus (NL), however there is minimaldata on long term outcomes in children exposed to maternalanti-Ro antibodies in utero. A previous pilot study utilising theResearch Registry for Neonatal Lupus (RRNL) raised concernregarding the development of autoimmune disease in childhood, however the numbers evaluated were small and the patientsstudied were young. This study was initiated to ascertain the current prevalence of autoimmune disease in NL children and theirunaffected siblings, and to evaluate whether fetal or maternal factors associated with the development of future autoimmunity.Materials and methods A retrospective cohort of family membersfrom the RRNL were contacted to evaluate for autoimmune disease. Follow-up questionnaires were completed which included35 items describing symptoms and diagnoses associated withautoimmunity in 138 cardiac NL children, 74 cutaneous NL children, and 134 unaffected siblings. Medical records were obtainedand evaluated from the patient's physicians to confirm diagnoses.Maternal diagnosis of systemic lupus and/or Sjogren's syndromeand fetal cardiac disease severity based on a previously describedseverity score were associated with postnatal autoimmune diseases using chi square and Mann-Whitney analyses.Results Seventeen (8.0%) of NL affected children developed anautoimmune disease at the time of follow up (mean age 11.6+/-9.0 years). These included 3 patients with SLE, 1 with JIA, 3with thyroid disease, 5 with psoriasis, 1 with IBD, 1 with uveitis,1 with UAS and 2 with type 1 DM. Six (4.5%) unaffected siblingsdeveloped an autoimmune disease (mean age 10.6+/-7.1 years),which included 1 with JIA, 1 with Sarcoidosis/ITP, 1 with Myasthenia Gravis/Celiac disease, 1 with psoriasis, 1 with UAS and1 with type 1 DM,. There was a significant association ofbetween having an autoimmune disease and having advancedheart block (11.0% vs. 4.2%, p = 0.03) and a trend towards anassociation with cardiac NL disease severity score (4.43+/-4.89 vs.2.58+/-4.24, p = 0.06). Mother's diagnosis of SLE or Sjogren'sdid not associate with the children's development of autoimmunedisease (p = 0.828).Conclusions Fetuses that develop advanced congenital heartblock as a manifestation of NL may be at greater risk for developing autoimmune diseases later in life. This could potentially relateto a genetic component that makes a Ro exposed fetus both moreprone to inflammatory effects of passive immunity and predisposes to future autoimmunity, independent of the mother's rheumatic disease status