Both comorbidities and differences in triglyceride-rich lipoprotein metabolism drive complication patterns in people with familial or multifactorial chylomicronemia syndromes (FCS, MCS), according to research published in The Journal of Clinical Endocrinology and Metabolism.
Researchers conducted an observational, national, multicenter study of people with FCS or MCS in order to compare pancreatic, cardiovascular, and diabetes-specific outcomes over a 10-year follow-up period.
Electronic health records from 4 tertiary referral lipid clinics in France were reviewed to identify patients. Those with a documented history of plasma triglyceride levels above 885 mg/dL were considered for inclusion. In total, 29 patients with FCS and 124 patients with MCS were matched with 413 controls.
Median follow-up for the FCS group was 11 years, with 2 recorded deaths; follow-up for the MCS group was 8 years, and no deaths were recorded.
The MCS group was primarily men (70.2% vs 41.4% in the FCS group). Patients in the FCS group were on average 11 years younger (34.3±13.6 years), and most were of a normal weight. Sixteen patients in the MCS group had a diabetes diagnosis prior to MCS diagnosis (12.9% vs no FCS patients).
Within the FCS group, 58.6% of patients had at least 1 hospital admission for acute hypertriglyceridemic pancreatitis (AHP) during the follow-up period, compared with only 19.4% of the MCS group (hazard ratio [HR], 3.6; 95% CI, 1.9-6.7; P <.01). Mean number of AHP episodes by life decade was 0.8 (±0.8) and 0.1 (±0.2) for the FCS and MCS groups, respectively.
All patients but 1 in the FCS group with AHP during follow-up had multiple hospitalizations, while only 15 of 124 patients in the MCS group had a history of “several recurrent AHP events” during follow-up.
More patients in the MCS group had at least 1 hospitalization for ischemic cardiovascular disease during the follow-up period compared with those in the FCS group (25% vs 10.3%; HR, 0.3; 95% CI, 0.1-1.0; P =.05). The trend for major adverse cardiovascular events was consistent over the 10-year follow-up (0.0% vs 7.3% in FCS and MCS, respectively).
Rates of diabetes diagnosis following FCS or MCS diagnosis were high and similar in both groups (24.1% vs 29.0%, respectively). Diabetes incidence was also high compared with the general population (odds ratio [OR], 22.8; 95% CI, 2.6-200.4 for FCS vs controls and OR, 30.3; 95% CI, 10.4-88.1 for MCS vs controls; P <.01 for both).
One study limitation of note was the inability of researchers to determine patient smoking patterns over the 10-year follow-up period due to the nature of the study design.
“These data illustrate the severity of such rare dyslipidemia,” the researchers concluded. “Both differences in [triglyceride]-rich lipoproteins and comorbidities in MCS vs FCS drive the occurrence of different patterns of complications.”
Disclosure: This clinical trial was supported by a grant from a pharmaceutical company. Please see the original reference for a full list of authors’ disclosures.
Belhassen M, Van Ganse E, Nolin M, et al. 10-year comparative follow up of familial versus multifactorial chylomicronemia syndromes. Published online November 21, 2020. J Clin Endocrinol Metab. doi:10.1210/clinem/dgaa838