Long-term management of relapsing anti-GPIHBP1 (Glycosylphosphatidylinositol-anchored high-density lipoprotein-binding protein 1) associated hypertriglyceridaemia: a case report and literature review
Abstract
Anti-GPIHBP1 syndrome is a rare, acquired cause of hypertriglyceridaemia, characterized by severe elevation of triglyceride that is refractory to standard lipid-lowering therapies. It is autoimmune in nature and demonstrates a significant response to immunosuppressive treatment. However, it has a chronic relapsing course and the role of maintenance immunosuppression in this syndrome remains unclear. We report a 46-year-old woman diagnosed with anti-GPIHBP1 syndrome following an initial presentation of acute pancreatitis. While her initial presentation and short-term induction response were previously reported, we would like to present her longitudinal follow-up, highlighting the long-term relapsing nature of the syndrome. She showed a good therapeutic response to intravenous rituximab infusions. Due to recurrent episodes of pancreatitis, maintenance immunosuppression with rituximab was commenced. Serial monitoring of CD19+ B-cell repopulation was used to guide the subsequent dosing timing as a hypothesis-generating strategy to aid in relapse prevention. During the latest follow-up, she remained in clinical and biochemical remission with proactive rituximab maintenance infusions. This single case observation suggests that patients with relapsing anti-GPIHBP1 syndrome might benefit from maintenance treatment with rituximab. Furthermore, regular monitoring of CD19+ B-cell repopulation may serve as a potential biomarker to guide retreatment, though it warrants further evaluation in more patients.
Related
- Analysis of reproductive traits associated with lipoprotein(a) deficiency in participants from the Pakistan Genome Resource
- Long-term survival in MIRAGE syndrome: Insights into systemic manifestations and management with review of literature
- Myroides bacteremia associated with wound myiasis: A case report and focused literature review.
- Novel Compound Heterozygous RECQL4 Pathogenic Variants and Associated Phenotypes in a Chinese Patient With Rothmund-Thomson Syndrome: Case Report and Literature Review.
- Congenital fibrovascular pupillary membrane with secondary glaucoma and associated fundus anomalies: a case report and literature review.
BibTeX
@article{em2026longterm,
title = {{Long-term management of relapsing anti-GPIHBP1 (Glycosylphosphatidylinositol-anchored high-density lipoprotein-binding protein 1) associated hypertriglyceridaemia: a case report and literature review}},
author = {Jun Min Em and Tharsini Sarvanandan and Jeyakantha Ratnasingam and Pavai Sthaneshwar and Masami Murakami and Soo Kun Lim},
journal = {Frontiers in Immunology},
year = {2026},
month = nov,
doi = {10.3389/fimmu.2026.1972947},
url = {https://www.frontiersin.org/articles/10.3389/fimmu.2026.1972947},
note = {CC BY 4.0},
}
RIS
TY - JOUR
TI - Long-term management of relapsing anti-GPIHBP1 (Glycosylphosphatidylinositol-anchored high-density lipoprotein-binding protein 1) associated hypertriglyceridaemia: a case report and literature review
AU - Jun Min Em
AU - Tharsini Sarvanandan
AU - Jeyakantha Ratnasingam
AU - Pavai Sthaneshwar
AU - Masami Murakami
AU - Soo Kun Lim
PY - 2026
DA - 2026/11/02
JO - Frontiers in Immunology
DO - 10.3389/fimmu.2026.1972947
UR - https://www.frontiersin.org/articles/10.3389/fimmu.2026.1972947
AB - Anti-GPIHBP1 syndrome is a rare, acquired cause of hypertriglyceridaemia, characterized by severe elevation of triglyceride that is refractory to standard lipid-lowering therapies. It is autoimmune in nature and demonstrates a significant response to immunosuppressive treatment. However, it has a chronic relapsing course and the role of maintenance immunosuppression in this syndrome remains unclear. We report a 46-year-old woman diagnosed with anti-GPIHBP1 syndrome following an initial presentation of acute pancreatitis. While her initial presentation and short-term induction response were previously reported, we would like to present her longitudinal follow-up, highlighting the long-term relapsing nature of the syndrome. She showed a good therapeutic response to intravenous rituximab infusions. Due to recurrent episodes of pancreatitis, maintenance immunosuppression with rituximab was commenced. Serial monitoring of CD19+ B-cell repopulation was used to guide the subsequent dosing timing as a hypothesis-generating strategy to aid in relapse prevention. During the latest follow-up, she remained in clinical and biochemical remission with proactive rituximab maintenance infusions. This single case observation suggests that patients with relapsing anti-GPIHBP1 syndrome might benefit from maintenance treatment with rituximab. Furthermore, regular monitoring of CD19+ B-cell repopulation may serve as a potential biomarker to guide retreatment, though it warrants further evaluation in more patients.
SN - 1664-3224
ER -