Steroid therapy still plays a crucial role and could serve as a bridge to the next promising treatments in patients with IgG4-related sclerosing cholangitis: Results of a Japanese nationwide study

Objective The acceptable duration of steroid therapy for patients with IgG4-sclerosing cholangitis (SC) has been under debate. Our aim is to clarify the feasible duration of steroid treatment. Design We retrospectively reviewed the data of patients with IgG4-SC and analyzed the following: biliary status during the steroid therapy, incidence of remission, relapse, relapse-free survival rate, and steroid-related complications (SRCs). Results Remission was achieved in 99.5% (763/767) of patients who received steroid therapy, while the remission rate dropped to 63.6% (78/129) of patients who did not receive it. Relapse was noted in 19.7% (151/763) of the patients who received steroid. Besides, relapse rate went up 38.4% (30/78) of the counterpart. Normalization of the serum total bilirubin and serum alkaline phosphatase levels were achieved at 2 weeks regardless of biliary drainage. Multivariate analysis identified younger onset, MST less than 3 years, immunosuppressant, and steroid cessation as independent risk factors for relapse. Steroid-free was achieved in the patients underwent MST only 3.4% over 54 months. SRCs were recorded in a total of 99 patients (12.9%) despite sufficient preemptive medications. Multivariate analysis identified history of malignancy and immunosuppressant as independent risk factors for SRCs. Conclusion Steroid therapy should be continued for no <3 years to reduce the risk of relapse, with use of preemptive measures taken around 5 years. The biliary drainage might not be mandatory. Steroid as 1st line therapy could serve as a bridge to further promising treatments.

Automated summary

This study aimed to clarify the feasible duration of steroid treatment for patients with IgG4-sclerosing cholangitis (SC). The results showed that steroid therapy significantly increased the remission rate but carried a risk of relapse. Younger age, shorter disease duration, use of immunosuppressants, and discontinuation of steroid medication were identified as independent risk factors for relapse. Preemptive measures can reduce the occurrence of steroid-related complications.