Year 2025 / Volume 117 / Number 6
Letter
Fatal outcome of a corticosteroid resistant IgG4-related autoimmune hepatitis and IgG4-primary sclerosing cholangitis

356-357

DOI: 10.17235/reed.2024.10310/2024

María del Carmen García Gavilán, Teresa Pereda Salguero, Susana López Ortega, Carlos Romero Gómez,

Abstract
33-year-old woman, previously hospitalized for self-limited migratory bile duct strictures, presented with jaundice three months after giving birth. Blood analysis revealed elevated levels of aspartate aminotransaminase 1064U/L, alanine aminotransaminase 1097U/L, gamma-glutamyl transferase 194U/L, alkaline phosphatase 284U/L, bilirubin 27mg/dL and prothrombin time of 19.3s. Magnetic resonance-cholangiopancreatography revealed intrahepatic bile duct dilation with a stenosis in the common hepatic duct, not detected on endoscopic retrograde cholangiopancreatography. Additionally, diffuse signal abnormalities were observed in the liver parenchyma on T2 sequences. An early liver biopsy showed moderate-severe interface hepatitis with IgG4-positive plasma cell infiltration (IgG4-PPC) of 8-20cells/HPF, hepatocellular ballooning and focal rosette formation, yielding 6 points of the simplified-score for autoimmune hepatitis and treatment with methylprednisolone was initiated. Despite treatment, there was no improvement after two weeks and the patient received rituximab as a rescue treatment, but three days later, developed candida sepsis with rapid progression to multiorgan failure, ultimately resulting in death.
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García Gavilán M, Pereda Salguero T, López Ortega S, Romero Gómez C. Fatal outcome of a corticosteroid resistant IgG4-related autoimmune hepatitis and IgG4-primary sclerosing cholangitis . 10310/2024


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Publication history

Received: 29/01/2024

Accepted: 16/04/2024

Online First: 30/04/2024

Published: 10/06/2025

Article Online First time: 92 days

Article editing time: 498 days


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