Pregnancy alters lipid metabolism, leading to elevation in plasma lipids, especially triglycerides (TG). Physiological changes may exacerbate underlying disorders of lipid metabolism, provoking major morbidity and mortality. We present the case of a 29yo nullipara with acute pancreatitis at 21/40 due to unmasked lipoprotein lipase deficiency (LLD).
Our patient had undifferentiated familial hyperlipidaemia pre-pregnancy (TG 2.1-6.2 mmol/L). Lipid-lowering medications were stopped in early pregnancy after an obstetrician advised they were not required. She presented to her local hospital at 21/40 with acute pancreatitis (lipase 195 U/L, pancreatic necrosis on MRI) and severe hypertriglyceridaemia (total cholesterol 28.9mmol/L, TG 91.1mmol/L) ultimately requiring admission to ICU at 27/40. She was then transferred to our unit for plasma exchange (PEX).
Plasma exchange resulted in a rapid fall in TGs (49.5mmol/L - 13.7mmol/L) and resolution of pancreatitis. TG levels gradually rose again and by the following week had returned to 33.7mmol/L. She underwent weekly PEX throughout pregnancy (target TG<40mmol/L) (Fig 1). Medical management included acipimox, gemfibrozil, and metformin. She followed a high-protein, low-fat, low-carbohydrate diet. She had no recurrence of pancreatitis and no obstetric complications. She had a vaginal birth of a healthy term female infant (3005g – 48th percentile). Postpartum lipid levels rapidly returned to baseline.
LLD is a rare autosomal-recessive disorder. Our patient had a mild phenotype outside of pregnancy, but a marked deterioration during pregnancy. Diet and medication were unable to prevent development of dangerously high TG levels but weekly PEX enabled good outcomes for both mother and baby.