Pamidimukkala Ujwala, Aitcheson Gabriella, Fatima Hala, Obaitan Itegbemie
Indiana University School of Medicine, Indianapolis, IN.
Division of Gastroenterology and Hepatology, Indiana University School of Medicine, Indianapolis, IN.
ACG Case Rep J. 2025 Apr 16;12(4):e01672. doi: 10.14309/crj.0000000000001672. eCollection 2025 Apr.
Immunoglobulin G4 (IgG4) sclerosing cholangitis is an immune-mediated fibroinflammatory disease of the biliary tree. It may be asymptomatic or cause abdominal pain, jaundice, or pruritus on presentation. While glucocorticoids and rituximab are regarded as initial treatment options, there is little guidance on the management of patients who either cannot tolerate these agents or are refractory to them. We discuss a case of IgG4 sclerosing cholangitis that required a novel treatment strategy of 1,000 mg oral mycophenolate mofetil twice daily and 375 mg/m rituximab infusions every 3 months to achieve disease control and limit adverse effects.
免疫球蛋白G4（IgG4）硬化性胆管炎是一种免疫介导的胆管树纤维炎性疾病。它可能无症状，或在发病时引起腹痛、黄疸或瘙痒。虽然糖皮质激素和利妥昔单抗被视为初始治疗选择，但对于那些不能耐受这些药物或对其耐药的患者的管理，几乎没有指导意见。我们讨论了一例IgG4硬化性胆管炎病例，该病例需要一种新的治疗策略，即每天口服两次1000毫克霉酚酸酯，并每3个月静脉输注375毫克/平方米的利妥昔单抗，以实现疾病控制并限制不良反应。
