Background/Purpose

Evidence suggests that both hydrocephalus and macrocephaly could be potential manifestations of neonatal lupus. In a recent study of 87 children born to mothers with anti-Ro antibodies (Boros et al., Arthritis Rheum, 2007), prevalence of hydrocephalus was high at 8.0% and mean head circumference was substantially larger than the age-matched normal values. Although up to 40% of women with SLE display anti-Ro antibodies, to date, no one has assessed the occurrence of hydrocephalus and/or macrocephaly in SLE offspring. Thus, in a large population-based study, we aimed to determine if children born to women with SLE have an increased risk of hydrocephalus and/or macrocephaly compared to children born to women without SLE.

Methods

The “Offspring of SLE mothers Registry (OSLER)” includes all women who had ≥1 hospitalization for delivery after SLE diagnosis, identified through Quebec’s universal healthcare databases (1989-2009). OSLER also includes a randomly selected control group of women, matched at least 4:1 for age and year of delivery, who did not have a diagnosis of SLE prior to or at the time of delivery. We identified children born live to SLE mothers and their matched controls, and ascertained hydrocephalus and macrocephaly based on ≥1 hospitalization or physician visit with a relevant diagnostic code, through to end of database follow-up. We performed multivariate logistic regression analyses, using generalized estimating equations, to adjust for maternal demographics and comorbidities, sex of child, and gestational diabetes.

Results

A total of 719 children were born to 509 women with SLE, and 8493 children were born to the 5824 matched controls. Compared to controls, children born to women with SLE showed only a slight trend towards more records of hydrocephalus and/or macrocephaly diagnosis [8 per 1000 persons (95% CI 4, 18) versus 6 per 1000 persons (95% CI 5, 8), difference 2 per 1000 persons (95% CI -3,13)]. Similarly there was only a slight trend towards younger age at time of hydrocephalus and/or macrocephaly diagnosis in SLE offspring versus controls [respectively 0.6 year (95% CI 0.2, 1.0) and 1.1 years (95% CI 0.5, 1.7)].  Of note, among the 6 cases of hydrocephalus and/or macrocephaly identified in SLE offspring, none had a diagnosis of cardiac conduction disturbance, suggesting no strong association with neonatal lupus. In multivariate analysis, the point estimate for the outcome was consistent with a trend for higher risk of hydrocephalus and/or macrocephaly in SLE offspring compared to controls, although the confidence interval was wide and precluded definitive conclusions (OR 1.37, 95% CI 0.58, 3.22).

Conclusion

Compared to children from the general population, there was a slight trend for higher frequency (and earlier age at diagnosis) of hydrocephaly and/or macrocephaly among children born to mothers with SLE, but the results do not strongly suggest an important increase in the risk of hydrocephalus and/or macrocephaly.

ACR Meeting Abstracts - https://acrabstracts.org/abstract/risk-of-hydrocephalus-andor-macrocephaly-in-children-born-to-mothers-with-sle/