Background <p>Intrahepatic cholestasis of pregnancy (ICP) is a pregnancy-specific liver disorder associated with pruritus and increased bile acids. While ursodeoxycholic acid (UDCA) is considered first-line therapy, optimal strategies for managing extreme, treatment-refractory cases remain inadequately defined. This case involves severe, early-onset ICP with an unusual and rapid clinical response to bezafibrate following failure of conventional therapy.</p> Case presentation <p>A 33-year-old woman, gravida 4 para 3, presented at 19 weeks’ gestation with generalized pruritus and was diagnosed with ICP. Despite escalation of UDCA to 2,000&#xa0;mg/day and sequential addition of rifampin and cholestyramine, bile acid concentrations peaked at 389 µmol/L, and liver transaminases remained markedly increased. Due to lack of improvement, bezafibrate was initiated at 23 weeks’ gestation. This resulted in gradual and sustained clinical and biochemical improvement with normalization of maternal liver function at 33 weeks’ gestation. At 36 weeks’ gestation, labor was induced, and a healthy male infant was delivered.</p> Conclusions <p>This case highlights a rare, severe, and treatment-refractory presentation of ICP. The marked improvement following bezafibrate supports its potential as a therapeutic consideration in similar high-risk cases. Further research is needed to evaluate its safety and efficacy in pregnancy.</p>

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Bezafibrate for severe refractory intrahepatic cholestasis of pregnancy: a case report and literature review

  • Erika Gandelsman,
  • Randa Taher,
  • Rinat Gabbay-Benziv,
  • Fadi Abu Baker

摘要

Background

Intrahepatic cholestasis of pregnancy (ICP) is a pregnancy-specific liver disorder associated with pruritus and increased bile acids. While ursodeoxycholic acid (UDCA) is considered first-line therapy, optimal strategies for managing extreme, treatment-refractory cases remain inadequately defined. This case involves severe, early-onset ICP with an unusual and rapid clinical response to bezafibrate following failure of conventional therapy.

Case presentation

A 33-year-old woman, gravida 4 para 3, presented at 19 weeks’ gestation with generalized pruritus and was diagnosed with ICP. Despite escalation of UDCA to 2,000 mg/day and sequential addition of rifampin and cholestyramine, bile acid concentrations peaked at 389 µmol/L, and liver transaminases remained markedly increased. Due to lack of improvement, bezafibrate was initiated at 23 weeks’ gestation. This resulted in gradual and sustained clinical and biochemical improvement with normalization of maternal liver function at 33 weeks’ gestation. At 36 weeks’ gestation, labor was induced, and a healthy male infant was delivered.

Conclusions

This case highlights a rare, severe, and treatment-refractory presentation of ICP. The marked improvement following bezafibrate supports its potential as a therapeutic consideration in similar high-risk cases. Further research is needed to evaluate its safety and efficacy in pregnancy.