Clinical Features, Differential Diagnosis and Treatment of IgG₄-Related Sclerosing Cholangitis

Abstract

The aim: To present the state-of-the-art of clinical features, differential diagnosis and treatment of IgG4-related sclerosing cholangitis.Key points: IgG4-sclerosing cholangitis is a fibrotic inflammatory disease affecting the intrahepatic and extrahepatic bile ducts. The clinical features of IgG4-sclerosing cholangitis are similar to those of primary sclerosing cholangitis, bile duct cancer and pancreatic cancer. More than one third of patients with IgG4-sclerosing cholangitis undergo surgery. Currently, there are no specific and sensitive methods to diagnose this disease. Increased serum IgG4 levels are observed in many other diseases. A fourfold increase in serum IgG4 levels is a more reliable marker, but this feature is found in only a small percentage of patients. The imaging of bile ducts usually reveals segmental or extended strictures with prestenotic dilatation and wall thickening. Glucocorticosteroids are the first-line therapy for induction and maintenance of disease remission. More than a half of patients develop relapses. Several studies have found an increased risk of malignant tumors. This review describes the clinical, laboratory, and instrumental features of IgG4-sclerosing cholangitis. Comparative evaluation of diseases manifestations versus primary sclerosing cholangitis and cholangiocarcinoma is presented along with options of therapy, prognosis and outcomes of the disease.Conclusion: IgG4-sclerosing cholangitis is a rare and difficult to diagnose disease that requires careful differential diagnosis with primary sclerosing cholangitis, bile duct cancer and pancreatic cancer. Despite its relatively benign course and efficacy of glucocorticosteroid therapy, the disease recurs frequently and has an unknown long-term outcome. Special attention is paid to the risk of malignant neoplasms in this group of patients, emphasizing the need for lifelong follow-up.