Treatment and prognosis of immunoglobulin G4-related hepatobiliary disease
10.3760/cma.j.issn.1007-3418.2018.06.004
- VernacularTitle: 免疫球蛋白G4相关肝胆疾病的治疗与转归
- Author:
Ling GONG
1
;
Junping SHI
Author Information
1. Department of Hepatology, Hangzhou Normal University Affiliated Hospital, Hangzhou 310015, China
- Publication Type:Journal Article
- Keywords:
Therapeutics;
Relapse;
Immunoglobulin G4-related hepatobiliary disease;
Immunoglobulin G4-related autoimmune pancreatitis;
Immunoglobulin G4-related sclerosing cholangitis
- From:
Chinese Journal of Hepatology
2018;26(6):411-414
- CountryChina
- Language:Chinese
-
Abstract:
Currently, there is no randomized controlled clinical trial of immunoglobulin (Ig) G4-related diseases in the world. Therefore, the best-known evidence-based medical treatment plan for this disorder is unavailable. The goal of IgG4-related hepatobiliary diseases treatment is to alleviate symptoms, prevent disease-related complications and fibrosis progression. A definite diagnosis is warranted before treatment. Hormonal therapy has become the basis of induction of remission in IgG4-related hepatobiliary disease. An initial prednisone dose is 30 ~ 40mg/d or 0.6 mg.kg-1.d-1 for 2 to 4 weeks, thereafter, gradually the dose is reduced within 2-3 months. Maintenance therapy with low-dose glucocorticoids hormone (prednisone 2.5 to 5.0 mg/d) is recommended for 1 to 3 years to prevent disease recurrence. In addition, immunosuppressive agents are equally effective, and in most cases, hormone combined immunosuppressive therapy may respond. Rituximab, a monoclonal antibody is a promising drug for treatment of this kind of diseases.
