Background: Hypertriglyceridemic acute pancreatitis (HAP) is the most serious complication of chylomicronemia (CM). The risk of HAP importantly increases during pregnancy. The main causes of CM include familial chylomicronemia syndrome (FCS), a rare autosomal recessive disorder and multifactorial chylomicronemia syndrome (MCS). The risk of HAP in MCS is much less established than in FCS. In this study, we compared the incidence and consequences of HAP during pregnancy in FCS women carrying two pathogenic variants in genes involved in the regulation of TG lipolysis vs women with severe MCS, most of them carrying at least one pathogenic FCS causing variant.
METHODS AND RESULTS: Pregnancies of women with history of chylomicronemia were retrospectively assessed in three Lipids clinics in France and Canada. Data collection was made through questionnaires and review of medical records. Comparison of the occurrence of HAP and pregnancy outcomes were performed in MCS vs FCS. 116 pregnancies were reviewed among 20 FCS (52 pregnancies) and 29 MCS (65 pregnancies) women. Both groups were comparable for age, BMI, maximum TG values and incidence of gestational diabetes. HAP during pregnancy affected a greater proportion of FCS women than MCS (42% vs 10%, p=0.01) and were more frequently recurrent in FCS (17% vs 5% p=0.02) regardless of the moment of the diagnosis. FCS women benefiting from a close medical and nutritional follow-up tended to have a lower HAP incidence (7% vs 39%, p=0.01). In both groups, the incidence of miscarriages and intra uterine growth retardation was comparable to the general population. However, the percentage of prematurity was elevated in both groups, particularly in FCS (56% vs 19%, p=0.01).
Conclusion: Pregnant women with chylomicronemia have 30-fold (MCS) to 120-fold (FCS) higher incidence of acute pancreatitis compared to the general population (0.35%). Close clinical monitoring during pregnancy is primordial to prevent HAP particularly in FCS.
