ObjectivesCardiac manifestations of neonatal lupus (NL) have been associated with significant morbidity and mortality; however, there is minimal information on long-term outcomes of affected individuals. This study was initiated to evaluate the presence of and the risk factors associated with cardiac dysfunction in NL after birth in multiple age groups to improve counselling, to further understand pathogenesis and to provide potential preventative strategies.MethodsEchocardiogram reports were evaluated in 239 individuals with cardiac NL: 143 from age 0–1 year, 176 from age >1–17 years and 64 from age >17 years. Logistic regression analyses evaluated associations of cardiac dysfunction at each age group with demographic, fetal and postnatal factors, using imputation to address missing data.ResultsCardiac dysfunction was identified in 22.4% at age 0–1 year, 14.8% at age >1–17 years and 28.1% at age >17 years. Dysfunction in various age groups was significantly associated with male sex, black race, lower fetal heart rates, fetal extranodal cardiac disease and length of time paced. In 106 children with echocardiograms at ages 0–1 year and >1–17 years, 43.8% with dysfunction at age 0–1 year were also affected at age >1–17 years, while the others reverted to normal. Of children without dysfunction at age 0–1 year, 8.9% developed new dysfunction between ages >1 and 17 years. Among 34 with echocardiograms at ages >1–17 years and >17 years, 6.5% with normal function at age >1–17 years developed dysfunction in adulthood.ConclusionsRisk factors in fetal life can influence cardiac morbidity into adulthood.Although limited by a small number of cases, cardiac dysfunction in the first year often normalises by later childhood. New-onset dysfunction, although rare, can occur de novo after the first year.
tion enrolled thus far and to demonstrate the breadth and potential value of the data generated by the new CARRA Registry. Methods We requested de-identified counts of several fields collected from the case report forms for subjects with SLE. Patients were eligible for enrollment in the new CARRA registry if they were diagnosed with SLE prior to the age of 18 and had either 1) a new diagnosis of SLE or 2) a flare of lupus nephritis within two years prior to the baseline visit. IRB approval was not required for this data request. Results To date, 184 patients (pts) have been enrolled; 156 (85%) are female. There are 46 black pts, 45 Hispanic pts, 47 white pts, 18 Asian pts and 16 pts were >1 race. Over half the pts have private health insurance (n=95, 52%) and 60 pts (33%) have Medicaid. Autoantibody positivity was prevalent: 175 pts (95%) were ANA positive, 109 (59%) dsDNA positive, and 87 (47%) anti-Smith positive. Positivity for anti-RNP, anti-Ro, anti-La, and APLs ranged from 15% to 51%. At the baseline visit, the mean Systemic Lupus Erythematosus Disease Activity Index (SLEDAI, n=166) score was 5.5±6.3, median=4 (range 0-37; IQR 0.25-8). The mean Systemic Lupus International Collaborating Clinic Damage Index (SLICC DI, n=150) score was 0.4, median=0 (range 0-7). Approximately one quarter of pts (n=50) were being treated for lupus nephritis at the time of the baseline visit. Manifestations of SLE at the baseline visit were varied (table 1) but serologic disease, mucocutaneous disease and active nephritis were the most prevalent. Conclusions Nearly 200 SLE pts have been enrolled in the new CARRA Registry to date. This is a multi-racial cohort with moderate disease activity and varied disease manifestations. Further enrollment will continue to build a robust data source to study disease course and outcomes in a pediatric SLE inception cohort. Acknowledgements The authors wish to acknowledge the Arthritis Foundation for ongoing financial support of CARRA and the CARRA Registry.
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