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Published on: February 28, 2013
Pancreatic and cardiometabolic complications of severe hypertriglyceridaemia
Bilal Bashir1,2,3, Maryam Ferdousi1,3, Paul Durrington1
1Faculty of Biology, Medicine and Health, University of Manchester.
Insights
Severe hypertriglyceridemia (SHTG) poses diagnostic and treatment challenges. Novel therapies targeting ApoC3 and ANGPTL3/8 show promise for managing SHTG and its complications.
Area of Science:
- Endocrinology
- Genetics
- Cardiology
Background:
- Severe hypertriglyceridemia (SHTG) presents diagnostic and therapeutic challenges.
- Familial chylomicronaemia syndrome (FCS) and multifactorial chylomicronaemia syndrome (MCS) are distinct forms of SHTG with varying complication risks.
- FCS remains underdiagnosed despite the FCS Score, with limited longitudinal data available.
Purpose of the Study:
- To explore the aetiopathogenesis and impact of SHTG and chylomicronaemia.
- To review cardiovascular and pancreatic complications associated with SHTG.
- To summarize novel pharmacological management options for SHTG.
Main Methods:
- Literature review of aetiopathogenesis, complications, and management of SHTG.
- Analysis of diagnostic challenges and current therapeutic strategies.
- Evaluation of emerging pharmacological targets like ApoC3 and ANGPTL3/8.
Main Results:
- SHTG, particularly FCS, is linked to acute pancreatitis risk, while MCS is associated with cardiovascular risks.
- Conservative management is standard for SHTG-induced pancreatitis; blood purification offers limited benefit.
- Conventional lipid-lowering drugs are largely ineffective, driving interest in antisense oligonucleotides (ASO) and short interfering RNA (siRNA) targeting ApoC3 and ANGPTL3/8.
Conclusions:
- Despite advances, SHTG diagnosis and treatment remain challenging due to rarity and heterogeneous phenotypes.
- Predictive models for complications and personalized treatment strategies are needed for FCS and MCS.
- National and international registries are crucial for improving disease understanding and identifying high-risk individuals.
Purpose Of Review:
This review endeavours to explore the aetiopathogenesis and impact of severe hypertriglyceridemia (SHTG) and chylomicronaemia on cardiovascular, and pancreatic complications and summarizes the novel pharmacological options for management.
Recent Findings:
SHTG, although rare, presents significant diagnostic and therapeutic challenges. Familial chylomicronaemia syndrome (FCS), is the rare monogenic form of SHTG, associated with increased acute pancreatitis (AP) risk, whereas relatively common multifactorial chylomicronaemia syndrome (MCS) leans more towards cardiovascular complications. Despite the introduction and validation of the FCS Score, FCS continues to be underdiagnosed and diagnosis is often delayed. Longitudinal data on disease progression remains scant. SHTG-induced AP remains a life-threatening concern, with conservative treatment as the cornerstone while blood purification techniques offer limited additional benefit. Conventional lipid-lowering medications exhibit minimal efficacy, underscoring the growing interest in novel therapeutic avenues, that is, antisense oligonucleotides (ASO) and short interfering RNA (siRNA) targeting apolipoprotein C3 (ApoC3) and angiopoietin-like protein 3 and/or 8 (ANGPTL3/8).
Summary:
Despite advancements in understanding the genetic basis and pathogenesis of SHTG, diagnostic and therapeutic challenges persist. The rarity of FCS and the heterogenous phenotype of MCS underscore the need for the development of predictive models for complications and tailored personalized treatment strategies. The establishment of national and international registries is advocated to augment disease comprehension and identify high-risk individuals.
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