The postnatal maturation of the immune system is poorly understood; as is its impact on illnesses afflicting preterm infants, such as bronchopulmonary dysplasia (BPD), a chronic lung disease. BPD affects up to 65% of very preterm infants and is characterized by inflammation and arrested development of alveoli and pulmonary blood vessels, leading to compromised lung function. The dysangiogenesis causes marked reduction in cross-sectional area, abnormal pulmonary vascular tone and elevated pressure in the pulmonary vasculature, resulting in pulmonary hypertension secondary to BPD (BPD-PH). BPD-PH occurs in up to 39% of severe BPD cases and represents its most significant complication. If not halted, BPD-PH can progress to right heart failure and up to 50% mortality. Currently, safe and effective treatments for BPD/BPD-PH are not available.
Investigating 51 preterm infants (blood obtained longitudinally at birth, day 1, weeks 1&2, and 36 weeks gestational age), 20 healthy term infants (birth, 3-16 weeks) and 5 healthy adults, we observed strong associations between type 2-polarization in circulating CD4+T cells and BPD (odds ratio up to 24). Unexpectedly, maternal magnesium sulfate therapy and delayed hepatitis B vaccination were associated with weaker type 2-polarization (e.g., up to 91% fewer type 2-polarized cells with hepatitis B-vaccination on d4-6 versus soon after birth), potentially conferring protection against BPD/BPD-PH. In a murine model of neonatal BPD/BPD-PH, blocking the type 2-mediators STAT6 or IL-4, IL-5 and/or IL-13 ameliorated lung inflammation and protected alveolar and vascular integrity: BPD-triggered increases in IL-1β and IL-13, and losses of pulmonary capillaries were prevented.
Our work advances knowledge on developmental immunology and its impact on early life diseases such as BPD. We identify maternal magnesium sulfate therapy, delayed hepatitis B vaccination and inhibition of type 2 mediators as promising therapeutic avenues that may lead to effective treatments for BPD/BPD-PH, relieving inflammation-driven suffering in our youngest patients.