Session Type: Abstract Submissions (ACR)
Background/Purpose: Several studies have evaluated mortality and short-term morbidity in neonatal lupus (NL), however there have been no substantive descriptions of the long term cardiac, rheumatologic, or neurodevelopmental outcomes in children exposed to maternal anti-Ro antibodies. This study was initiated to ascertain the prevalence of these outcomes in NL children and their unaffected siblings, and to evaluate whether certain fetal echocardiographic risk factors or pacemaker placement associates with long term morbidity.
Methods: The study utilizes a retrospective cohort of family members from the Research Registry for Neonatal Lupus (RRNL). Follow-up questionnaires were completed for 75 cardiac NL children, 35 cutaneous NL children, and 74 unaffected siblings. The questionnaires focus on symptoms associated with rheumatic and cardiac diseases, and information on pacemakers, developmental milestones, medical diagnoses and medications. Records from the RRNL were reviewed for fetal echocardiographic data.
Results: Of 184 total respondents, 52 children (28%) were age 0-5, 45 (24%) were 5-10, 30 (16%) were 10-15, 32 (17%) were 15-20, and 25 (14%) were > 20 years old. Among the 75 cardiac NL cases, 64 (85%) had 3rd degree heart block, 3 (4%) 2nd degree, 5 (7%) 1stdegree, and 3 (4%) isolated cardiomyopathy. 53 (79%) of the 67 with advanced block were paced, 43% of whom had at least one replacement. 15 (20%) cardiac NL cases were reported as ever having “heart failure” and 31 (41%) “an enlarged heart.” Median age was significantly higher in those reporting an enlarged heart (p=0.016), and those needing a pacemaker and replacement (p=.001 and 0.003, respectively). Of 37 cardiac NL children > 10 yrs, 19% required cardiac medications (digoxin, ACE inhibitor, or beta blocker), vs. 5% < 10 yrs (p=0.086). Fetal echocardiographic dilated cardiomyopathy and valvular disease were positively associated with number of pacemaker replacements (p=0.044 and 0.008). Of the total 110 NL patients, 1 developed JIA, 1 psoriasis/iritis, 1 IBD, and 3 thyroid disease. Of the 74 unaffected siblings, 1 developed 1 RA, and 1 sarcoidosis. Autism was diagnosed in 3 (3%) NL children and 2 (3%) healthy siblings. 5 (5%) and 6 (8%) NL and unaffected children respectively were taking medications for ADHD. Hydrocephalus was diagnosed after birth in 6 (5%) NL children, 3 requiring shunts. In 13 (12%) and 2 (3%) NL and unaffected children, delays in motor milestones were reported (p=0.027). 41 (37%) NL children had ever been considered small in height or weight for age vs. 14 (19%) healthy siblings (p=0.005). However, only 12 (11%) NL children were still considered small at the time of the questionnaire vs. 9 (12%) of the unaffected siblings.
Conclusion: Older age is associated with a reported history of heart enlargement, greater need for pacing, and cardiac medications. This supports the requirement for continued intensive long term cardiac evaluation in affected children. Risks exist in NL for delayed motor milestones, and transient failure to thrive. This further suggests the need for comprehensive surveillance, perhaps with a multidisciplinary protocol at a center familiar with these children.
P. M. Izmirly,
J. P. Buyon,
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ACR Meeting Abstracts - https://acrabstracts.org/abstract/long-term-outcomes-in-neonatal-lupus/