[Research status and challenges in immunoglobulin G4-related hepatobiliary disease].

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.

[Histopathological features and pathological diagnosis of IgG4-related hepatobiliary diseases].

IgG4-related diseases (IgG4-RD) can affect multiple organs, including liver and bile ducts, and manifested as IgG4-related sclerosing cholangitis, IgG4-related liver disease, and IgG4-related autoimmune hepatitis. IgG4-RD has common pathogenesis and histopathological features. A histopathological examination is very important for the diagnosis of IgG4-RD. This article reviews the histopathological features and pathological diagnosis of IgG4-RD and IgG4- related hepatobiliary diseases.

[Diagnosis and differential diagnosis of immunoglobulin G4-related hepatobiliary disease].

Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated disease that share common pathologic, serologic and clinical features. IgG4- RD may include inflammatory pseudotumor, IgG4-related autoimmune hepatitis, and type 1 autoimmune pancreatitis mainly involving liver and clinically classified into three types. IgG4-related sclerosing cholangitis is a rare disease. It is frequently present in association with type 1 autoimmune pancreatitis, so it needs to be distinguishing from primary sclerosing cholangitis.

[Treatment and prognosis of immunoglobulin G4-related hepatobiliary disease].

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.

Treatment and prognosis of immunoglobulin G4-related hepatobiliary disease / 中华肝脏病杂志

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.

Diagnosis and differential diagnosis of immunoglobulin G4-related hepatobiliary disease / 中华肝脏病杂志

Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated disease that share common pathologic, serologic and clinical features. IgG4- RD may include inflammatory pseudotumor, IgG4-related autoimmune hepatitis, and type 1 autoimmune pancreatitis mainly involving liver and clinically classified into three types. IgG4-related sclerosing cholangitis is a rare disease. It is frequently present in association with type 1 autoimmune pancreatitis, so it needs to be distinguishing from primary sclerosing cholangitis.

Histopathological features and pathological diagnosis of IgG4-related hepatobiliary diseases / 中华肝脏病杂志

IgG4-related diseases (IgG4-RD) can affect multiple organs, including liver and bile ducts, and manifested as IgG4-related sclerosing cholangitis, IgG4-related liver disease, and IgG4-related autoimmune hepatitis. IgG4-RD has common pathogenesis and histopathological features. A histopathological examination is very important for the diagnosis of IgG4-RD. This article reviews the histopathological features and pathological diagnosis of IgG4-RD and IgG4- related hepatobiliary diseases.

Research status and challenges in immunoglobulin G4-related hepatobiliary disease / 中华肝脏病杂志

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.