Successful Use of a Novel High-Dose Mycophenolate Mofetil and Rituximab Regimen for Progressive IgG4 Sclerosing Cholangitis With Multisystemic Involvement

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/m² rituximab infusions every 3 months to achieve disease control and limit adverse effects.