Authors
Ayano Yamaguchi, Naoya Teramoto, Suzuka Watanabe, Hiyori Kaneko, Saori Kami, Chihiro Hiraga, Tetta Sato, Makoto Miyabayashi, Kazuto Aono, Katsushi Igarashi, Takahiro Ishikawa, Masatsune Ogura, Junji Kobayashi, Takashi Yamaguchi, Kazuya Miyashita, Katsuyuki Nakajima, Koutaro Yokote, Kei Ikeda, Masami Murakami, Yoshiro Maezawa
Published in
Journal of clinical lipidology. Jun 15, 2026. Epub Jun 15, 2026.
Abstract
Glycosylphosphatidylinositol-anchored high-density lipoprotein binding protein 1 (GPIHBP1) autoantibody syndrome is a rare autoimmune cause of severe hypertriglyceridemia and acute pancreatitis.
We report 2 patients who achieved long-term remission through distinct therapeutic approaches.
We report 2 patients who achieved long-term remission through distinct therapeutic approaches.
Case 1 received prednisolone, azathioprine, and hydroxychloroquine, maintaining remission and experienced an uneventful pregnancy while continuing hydroxychloroquine monotherapy for 4 years. Case 2, who suffered recurrent pancreatitis, was treated with rituximab and achieved sustained remission without maintenance therapy for 3 years.
Epitope mapping identified distinct antibody epitopes in the 2 cases. The antibody from Case 1 targeted the Ly6/uPAR (LU) domain of GPIHBP1, whereas the antibody from Case 2 recognized an epitope involving not only the LU domain but also the region bordering the acidic domain.
This is the first report demonstrating successful long-term remission of GPIHBP1 autoantibody syndrome, including during pregnancy. The clinical significance of epitope heterogeneity among GPIHBP1 autoantibodies remains to be clarified.
PMID:
42420131
Bibliographic data and abstract were imported from PubMed on 09 Jul 2026.
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