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.
ABSTRACT
IgG4-associated hepatobiliary diseases are group of autoimmune diseases characterized by lymphoplasmacytic infiltrates with an elevated serum IgG4 levels, affecting pancreas and biliary tract. In addition, it mainly includes IgG4-related sclerosing cholangitis, IgG4-related autoimmune pancreatitis and IgG4-related autoimmune hepatitis. An accurate diagnosis helps to avoid unnecessary surgery. Notably, an early diagnosis and treatment can improve the prognosis and enhance the quality of life. This review will focus on research advances and difficulties encountered in the study of IgG4 related hepatobiliary diseases.
ABSTRACT
Immunoglobulin G4-related disease (IgG4-RD)is an immune-mediate disease with common specific pathology,serological,and clinical features. IgG4-related autoimmune pancreatitis,hepatopathy and sclerosing cholangitis have characteristics of the focal or diffuse swelling of affected organs and tissues,elevated serum concentration of IgG4, rich IgG4 positive lymphoplasmacytic infiltration,different degrees of storiform pattern of fibrosis and obliterative phlebitis. They also have special characteristics in clinical manifestations,diagnosis and treatment. IgG4-RD generally responds to glucocorticoid with excellent prognosis,but is easily recurrent. This article summarized the advances in pathogenesis, diagnosis and treatment of IgG4-related autoimmune pancreatitis,hepatopathy and sclerosing cholangitis.