Relapsing immunoglobulin G4-related sclerosing cholangitis during maintenance treatment with low-dose steroids: a case report

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.

Keywords: Immunoglobulin G4-related sclerosing cholangitis (IgG4-SC); case report; immunoglobulin G4-related disease (IgG4-RD); relapse; steroid.

Figure 1

A timeline showing the disease course of this case.

Figure 2

MRI findings at the time of first attack and disease relapse in this patient. (A,B) Images before steroid therapy at the first attack. (A) Diffuse enlargement of pancreas with delayed enhancement (red arrows) and rim-like enhancement; dilated intrahepatic bile duct (black arrows). (B) Circular-symmetrical wall thickening in the lower common bile duct (white arrow). (C,D) Images after steroid therapy at the first attack. (C) Mild enlargement of pancreas with delayed enhancement (red arrows). (D) Thickened lower common bile duct wall with no significant change compared to previous images (white arrow). (E,F) Images before steroid therapy at the disease relapse. (E) Swollen pancreas morphology with no significant change compared to the previous images (red arrows), dilated intrahepatic bile duct (black arrows). (F) Thickened upper segment of common bile duct wall (white arrow), dilated intrahepatic bile duct (black arrows). MRI, magnetic resonance imaging.

Figure 3

MRCP findings at the first attack and disease relapse in this patient. (A,B) Images before (A) and after (B) steroid therapy at the first attack. (A) Dilated intrahepatic bile duct and common bile duct (white frame) with an inner diameter of about 1.3 cm, narrowed lower common bile duct (red frame), and obscure pancreatic duct (white arrows). (B) Improvement of dilated intrahepatic bile duct and common bile duct with a diameter of about 0.9 cm, improvement of narrowed low common bile duct (red frame), and clear pancreatic duct (white arrows). (C,D) Images before (C) and after (D) steroid therapy at the disease relapse. (C) Dilated intrahepatic bile duct (white frame), narrowed hilar bile duct and lower common bile duct (red frames) with a diameter of about 0.7 cm, and obscure pancreatic duct (white arrows). (D) Significant improvement of narrowed hilar bile duct and lower common bile duct (red frame), and clear pancreatic duct (white arrows). MRCP, magnetic resonance cholangiopancreatography.

Figure 4

An overview of disease relapse rates in IgG4-RD patients after steroid therapy alone. IgG4-RD, immunoglobulin G4-related disease.