Abstract
BACKGROUND: Immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) often has a good response to initial steroid therapy, but a high relapse rate during follow-up. Knowledge about the predictors and treatment strategy of relapsing IgG4-SC is of great significance. CASE DESCRIPTION: In this paper, we reported that a 57-year-old male was diagnosed with IgG4-SC accompanied by type 1 autoimmune pancreatitis (AIP) at the first onset of his diseases and had a good response to steroid therapy. However, during low-dose steroids maintenance therapy, IgG4-SC relapsed with clinical presentations related to severe bile duct stricture, but improved rapidly after re-administration of full-dose steroids, accompanied by resolution of jaundice, improvement of intrahepatic and extrahepatic bile duct stricture, and gradual recovery of liver function. At the last follow-up in December 2021, he was still stable with methylprednisolone tablets at 4 mg/day. CONCLUSIONS: IgG4-SC is likely to relapse in patients who have high serum IgG4 level at initial onset and receive low-dose steroids maintenance treatment. The predictors of disease relapse also include steroids interruption, more severe bile duct stricture, long duration from diagnosis to treatment, history of allergy, and high serum tumor necrosis factor-alpha (TNF-alpha) and soluble interleukin-2 receptor (sIL-2R) levels. Re-administration or up-dose of steroids, immunosuppressors, and rituximab are effective for treating relapsing disease.