Treatment: Immunomodulatory Drugs

Abstract

The Japanese clinical diagnostic criteria for IgG4-related sclerosing cholangitis (SC) established in 2012 are useful in the diagnosis of IgG4-related cholangiopathy [1]. IgG4-related SC, a distinctive type of cholangitis of unknown etiology, is characterized by increased serum levels of IgG4 and massive infiltration of IgG4-positive plasma cells with extensive fibrosis in the wall of the bile duct. Its cholangiographic features are similar to those of primary sclerosing cholangitis (PSC), pancreatic cancer, and cholangiocarcinoma. However, it has been reported that about approximately 60–85% of patients with type 1 autoimmune pancreatitis (AIP) have associated with IgG4-SC [1–5]. The steroid responses and the prognoses of IgG4-SC differ from those of patients with PSC, which suggests different pathologies. IgG4-SC responds well to steroid therapy, and the recommended first-line treatment for IgG4-SC is corticosteroid therapy. Unfortunately, despite the high initial remission rates, 15–60% of patients will develop disease relapse either after cessation of steroid therapy or during the weaning of the steroid dose [6–9]. In Japan, to prevent relapses in type 1 AIP (including IgG4-SC), many patients are advised to continue daily low-dose prednisolone (2.5–10 mg) for months to years following induction of remission. However, this maintenance of steroid therapy has its advantages and disadvantages. Recently, the outcome of a Japanese randomized controlled study regarding maintenance steroid therapy was reported [10]. In general, IgG4-SC is treated with steroids, immunomodulatory drugs, or rituximab. Herein, we discuss the treatment of IgG4-SC, focusing on immunomodulatory agents.