Relapse of IgG4-related sclerosing cholangitis after steroid therapy: image findings and risk factors.

OBJECTIVES

To compare imaging data from IgG4-related sclerosing cholangitis (IgG4-SC) obtained at initial attack and at relapse, and to determine risk factors predicting relapse of IgG4-SC after steroid therapy.

METHODS

Twenty-three patients with relapsed IgG4-SC and 36 non-relapsed patients were included. Computed tomography, magnetic resonance cholangiography, and endoscopic retrograde cholangiography were used to determine biliary and extrabiliary involvement. Images taken at the time of the initial attack were compared with those taken at relapse. Risk factors were determined by comparing clinical and imaging factors in the relapse and non-relapse groups.

RESULTS

In comparison to the initial attack, relapsed IgG4-SC was characterised by more frequent extrapancreatic and multiple bile duct strictures, increased bile duct segment involvement, thicker bile duct walls, and a less frequent association with autoimmune pancreatitis (AIP) (P ≤ 0.016). Compared with non-relapse, relapse was significantly associated with less frequent use of maintenance steroid therapy, more frequent extrapancreatic and multiple bile duct strictures, increased bile duct segment involvement, and thicker bile duct walls during the initial attack (P ≤ 0.021).

CONCLUSIONS

Relapsed IgG4-SC presented with a more aggressive appearance on imaging than the initial attack. The degree of bile duct involvement and use of maintenance steroid therapy were associated with relapse of IgG4-SC.