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
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.
ABSTRACT
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.
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
Objective@#To compare and analyze patient’s general condition, laboratory testing and therapeutic responses of isolated immunoglobulin G4- related sclerosing cholangitis (IgG4-SC) and immunoglobulin G4 sclerosing cholangitis combined autoimmune pancreatitis (IgG4-SC/AIP).@*Methods@#A retrospective study was conducted on IgG4-SC patients who attended outpatient and inpatients department of our hospital from April 2014 to March 2018 and their demographic characteristics, laboratory testing, and therapeutic responses were statistically analyzed. Normal distribution of continuous variables was compared with t-test, non-normal distribution of continuous variables was compared using the Mann-Whitney U test, and the categorical variables were compared with χ 2 test.@*Results@#29 IgG4-SC patients were included, including 19-isolated IgG4-SC and 10 IgG4-SC combined AIP (IgG4-SC/AIP). The average age of onset in the isolated IgG4-SC group was (46.06±19.03) years which was lower than IgG4-SC/AIP group (62.60±15.11), t = -2.360, P < 0.05. The median IgG4 in IgG4-SC/AIP patients is higher than that in isolated IgG4-SC, respectively 10.87 (3.73 ~ 20.13) and 3.14 (2.37 ~ 4.78)g/L(U = 159.000, P < 0.05). IgG4/IgG ratio is higher in IgG4-SC/AIP, than that in isolated IgG4-SC, respectively 0.62(0.23 ~ 0.86) and 0.16(0.10 ~ 0.21), U = 130.000, P < 0.05. Liver cirrhosis was more common in isolated IgG4-SC group (47%) than the IgG4-SC/AIP group (0), χ 2 = 9.637, P < 0.05. The median biochemical response time of isolated IgG4-SC group was 3.00 (2.00 to 4.00) months, which was longer than 1.00 (1.00 to 1.25) months of IgG4-SC/AIP group, U = 30.000, P < 0.05. The biochemical recurrence rate of isolated IgG4-SC group was 32%, which was lower than that of IgG4-SC/AIP (χ 2 = 6.461, P < 0.05).@*Conclusion@#Serum IgG4 level and IgG4/IgG ratio were higher in patients with IgG4-SC/AIP group, and therapeutic responses in isolated IgG4-SC patients were worse than that of IgG4-SC/AIP patients. The efficacy of glucocorticoid monotherapy and immunosuppressive agents combined with glucocorticoid therapy demonstrated no considerable difference in IgG4-SC patients.
ABSTRACT
IgG4-relaed hepatobiliary diseases (IgG4-HBD) are the hepatobiliary manifestations of IgG4-related disease, a multisystem fibro-inflammatory disorder. Previous studies on the pathogenesis of genetics and immunology have provided significant assistance in understanding the disease, rational diagnosis and treatment, but there are still many unknowns and challenges. The current research progress summarizes several factors influencing fibrosis and inflammation in the pathogenesis of disease.
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.