Kubota Kensuke, Kamisawa Terumi, Nakazawa Takahiro, Tanaka Atsushi, Naitoh Itaru, Takikawa Hajime, Unno Michiaki, Kawa Shigeyuki, Masamune Atsushi, Nakamura Seiji, Okazaki Kazuichi
Endoscopic Unit, Yokohama City University Hospital, Yokohama, Japan.
Department of Internal Medicine, Tokyo Metropolitan, Komagome Hospital, Tokyo, Japan.
J Hepatobiliary Pancreat Sci. 2022 Aug;29(8):884-897. doi: 10.1002/jhbp.1157. Epub 2022 May 25.
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.
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).
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.
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.
IgG4 硬化性胆管炎（SC）患者类固醇治疗的可接受疗程一直存在争议。我们的目的是明确类固醇治疗的可行疗程。
我们回顾性分析了 IgG4-SC 患者的数据，并分析了以下内容：类固醇治疗期间的胆道状况、缓解率、复发率、无复发生存率以及类固醇相关并发症（SRCs）。
接受类固醇治疗的患者中有 99.5%（763/767）实现缓解，而未接受治疗的患者缓解率降至 63.6%（78/129）。接受类固醇治疗的患者中有 19.7%（151/763）出现复发。此外，未接受类固醇治疗的患者复发率上升至 38.4%（30/78）。无论是否进行胆道引流，血清总胆红素和血清碱性磷酸酶水平在 2 周时均恢复正常。多因素分析确定发病年龄较轻、病程小于 3 年、使用免疫抑制剂以及停用类固醇是复发的独立危险因素。仅接受手术治疗（MST）的患者在 54 个月内仅有 3.4%实现无类固醇治疗。尽管采取了充分的预防措施，仍有 99 例患者（12.9%）出现 SRCs。多因素分析确定恶性肿瘤病史和使用免疫抑制剂是 SRCs 的独立危险因素。
类固醇治疗应持续不少于 3 年以降低复发风险，并在约 5 年时采取预防措施。胆道引流可能并非必需。类固醇作为一线治疗可作为进一步有效治疗的桥梁。
