Advances in diagnosis and treatment of IgG4-related hepatobiliary and pancreatic diseases / 临床肝胆病杂志

ABSTRACT

IgG4-related hepatobiliary and pancreatic diseases are a part of the IgG4-related disease multiorgan fibroinflammatory disorder, including IgG4-related autoimmune pancreatitis, IgG4-related sclerosing cholangitis, and IgG4-related hepatic involvement. The main pathological features include IgG4 + plasma cell/lymphocyte infiltration, storiform fibrosis, obliterative phlebitis, and eosinophil infiltration. The diagnosis of this disease is often based on the comprehensive diagnostic criteria for IgG4-related diseases and organ-specific diagnostic criteria. However, it is difficult to differentiate IgG4-related hepatobiliary and pancreatic diseases from neoplastic diseases, and novel diagnostic biomarkers are expected to improve the sensitivity and specificity of diagnosis. To date, glucocorticoids remain the first-line drug for this disease, and biological agents, especially anti-CD20 monoclonal antibody, may be an alternative therapy for patients with corticosteroid contraindication/intolerance or recurrent/refractory disease.