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Successful Long-Term Remission of Hyperchylomicronemia Caused by GPIHBP1 Autoantibodies: with Insights into Antibody Epitope Effects
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DOI：10.1016/j.jacl.2026.06.009.png)
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Abstract
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• Two patients with GPIHBP1 autoantibody syndrome achieved sustained remission for more than four years using distinct immunomodulatory strategies. • Hydroxychloroquine monotherapy maintained long-term remission in Case 1, allowing an uneventful pregnancy and delivery. • Rituximab induced durable remission without maintenance therapy in Case 2, who had recurrent acute pancreatitis. • Epitope analysis revealed different antibody targets: the LU domain alone in Case 1 and the linker region between acidic and LU domains in Case 2.
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