Course of Pregnancies and Occurrence of Acute Pancreatitis in Women with Chylomicronemia.

Objective: Chylomicronemia is characterized by extreme hypertriglyceridemia (triglycerides values >10 mmol/L). It may be caused by bi-allelic combination of pathogenic variant (familial chylomicronemia syndrome (FCS)) or by genetic susceptibility combined with comorbidities and environmental factors (multifactorial chylomicronemia syndrome (MCS)). Acute pancreatitis (AP) is the most serious complication of CM. In the general population, the prevalence of AP during pregnancy is estimated to be <0.35%. As triglycerides levels significantly increase during pregnancy, it may affect the course of pregnancy and further increase the risk of AP in women with chylomicronemia.

Methods: 116 pregnancies involving 49 European and North American women with history of chylomicronemia (20 FCS, 29 MCS) were retrospectively reviewed. The occurrence of AP, the course of pregnancy, fetal development and delivery were evaluated.

Results: 42% of FCS and 10% of MCS women experienced at least one AP episode during pregnancy (p=0.01). Compared to MCS, women with FCS presented a higher percentage of pregnancies with AP (17% vs 5%, p=0.02). Among all reviewed pregnancy-related AP, 56% occurred in primigravida FCS women compared to 0% in MCS. Premature deliveries were elevated in both groups although more frequent in FCS (56%) vs MCS (19%) (p=0.01). The percentage of miscarriages (11.8% vs 10.7%) and fetal failure to thrive (5.9% vs 9.2%) were not significantly different between the two cohorts.

Conclusions: In this study, pregnant women with chylomicronemia had 30-fold (MCS) to 120-fold (FCS) higher occurrence of AP compared to the general population. Chylomicronemia per se does not seem to influence fetal development.