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

• Published in: Journal of hepato-biliary-pancreatic sciences Vol. 29; no. 8; pp. 884 - 897
• Main Authors: Nakazawa, Takahiro, Takikawa, Hajime, Unno, Michiaki, Masamune, Atsushi, Okazaki, Kazuichi, Sawai, Shigeaki, Goto, Takuma, Otsuka, Masayuki, Tamano, Masaya, Koizumi, Mitsuhito, Hiasa, Yoichi, Kawabe, Naoto, Asano, Yukio, Watanabe, Hiroyuki, Toya, Daishu, Ohira, Hiromasa, Mukai, Tsuyoshi, Iwata, Keisuke, Shimizu, Shogo, Suetsugu, Jun, Tsuji, Keiji, Ishida, Ryoko, Ito, Masanori, Araki, Masahiro, Sakamoto, Yasunari, Takai, Satoshi, Ikeya, Shinichi, Yamada, Takanori, Ichiki, Yasunori, Kitada, Hideki, Hifumi, Michio, Kurosaki, Masayuki, Sumi, Hajime, Hayashi, Katsumi, Harada, Ryo, Nakamura, Shinichiro, Ito, Tetsuya, Oura, Kyoko, Masaki, Tsutomu, Shimokawahara, Naoto, Mizushima, Ichiro, Naganuma, Makoto, Murata, Miki, Nishio, Akiyoshi, Teratani, Takuma, Matsubara, Shohei, Azemoto, Ryousaku, Kamata, Ken, Kurosu, Takahiro, Fujita, Jun, Seki, Hideyuki, Ueda, Yasuhiro, Fukumoto, Takumi, Uchida, Kazushige, Ochiai, Toshimasa, Ishida, Etsuji, Mori, Hideaki, Sakagami, Junichi, Seno, Hiroshi, Naitoh, Itaru, Aoki, Toshinori, Mitoro, Akira, Yoshiji, Hitoshi, Yoshida, Motoyuki, Hayasaka, Kenji, Amano, Yuji, Terai, Shuji, Nakajima, Michiko, Yamahana, Junya, Iwatsu, Shinichi, Kato, Yuji, Horiguchi, Shigeru, Yagi, Takahito, Okada, Hiroyuki, Hirao, Motohiro, Imamura, Haruo, Shinobi, Tetsuya, Ryozawa, Shomei, Miyake, Tatsuya, Kohge, Naruaki, Tobita, Hiroshi, Joshita, Satoru, Kawaguchi, Shinya, Ohno, Kazuya, Yagi, Minami, Katanuma, Akio, Koike, Kazuhiko, Kagawa, Tatehiro, Azuma, Seishin, Kobayashi, Katsumasa, Asano, Toru, Taniguchi, Hideaki, Yasuda, Ichiro, Oe, Shinji, Harada, Masaru, Kurata, Masanao, Kawaji, Yuki, Kaino, Miyuki, Sakaida, Isao
• Format: Journal Article
• Language: English
• Published: Japan Wiley Subscription Services, Inc 01-08-2022
• Subjects: Cholangitis; IgG4‐related sclerosing cholangitis; Multivariate analysis; relapse; steroid; steroid related complications, limitation of steroid; Steroids; IgG4-related Sclerosing Cholangitis; steroid related complications, limitation of steroid; relapse; steroid
• ISSN: 1868-6974; 1868-6982
• DOI: 10.1002/jhbp.1157

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.
The acceptable duration of steroid therapy for IgG4‐sclerosing cholangitis remains debated. Kubota et al conclude that steroid therapy should be continued at least 3 years to reduce the risk of relapse, with preemptive measures taken around 5 years. Steroids as first‐line therapy could serve as a bridge to further promising treatments.