Diagnosis of isolated hilar-/extrahepatic-type IgG-4-related sclerosing cholangitis can be increased by improved recognition of this condition—A Japanese multicenter analysis

IF 3.2 3区 医学 Q2 GASTROENTEROLOGY & HEPATOLOGY
Kensuke Kubota, Eisuke Iwasaki, Takuya Ishikawa, Masaki Kuwatani, Mamoru Takenaka, Takuji Iwashita, Atsuhiro Masuda, Tsukasa Ikeura, Akira Nakamura, Atsushi Tanaka, Hiroyuki Isayama, Yoshiki Hirooka, Kenji Hirano, Shomei Ryozawa, Takeshi Ogura, Toshio Fujisawa, Yusuke Kurita, Kazuhiro Kikuta, Nobuhiko Hayashi, Atsushi Masamune, Ichiro Yasuda
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Abstract

Background

Patients with isolated IgG4-related sclerosing cholangitis (IgG4-SC) often undergo unnecessary resection. The aim of this study was to validate the revised Japanese diagnostic criteria for isolated IgG-4-SC and to improve awareness about this condition in the population.

Methods

This was a Japanese retrospective multicenter study. We focused on the data and diagnostic yield obtained using the Japanese diagnostic criteria published initially in 2012 and revised later in 2020 for the diagnosis of isolated IgG4-SC.

Results

Patients with isolated IgG4-SC could be classified into two groups based on the primary location of the lesion: the hilar type (n = 40) and the extrahepatic type (n = 13). In total, 10 patients with the hilar type had undergone unnecessary resection. The revised 2020 criteria are useful for the diagnosis of extrahepatic lesions, which are not included in the 2012 criteria. The need for a steroid trial was reduced from 37.7% when the diagnosis was based on the 2012 criteria to 7.6% when the diagnosis was based on the revised 2020 criteria. The diagnostic specificity also improved from 58.5% for the 2012 criteria to 88.7% for the revised 2020 criteria.

Conclusion

Our validation of the 2020 criteria for the diagnosis of IgG4-SC could contribute to avoiding unnecessary resection in patients with isolated IgG4-SC, which can be classified into the hilar and extrahepatic types. The 2020 criteria can enhance the diagnosis rate of isolated IgG4-SC and uncover this tough-to-diagnose entity based on inclusion of the imaging findings and decrease the dependence on a steroid trial.

Abstract Image

提高对孤立性肝门/肝外型 IgG-4 相关硬化性胆管炎的识别能力可提高诊断率--日本多中心分析。
背景:孤立性 IgG4 相关硬化性胆管炎(IgG4-SC)患者通常会接受不必要的切除手术。本研究旨在验证日本修订后的孤立性 IgG-4-SC 诊断标准,并提高民众对该病的认识:这是一项日本多中心回顾性研究。方法:这是一项日本的回顾性多中心研究,我们重点研究了使用最初于 2012 年发布、后于 2020 年修订的日本诊断标准诊断孤立性 IgG4-SC 所获得的数据和诊断率:根据病变的原发部位,孤立性 IgG4-SC 患者可分为两组:肝门型(40 例)和肝外型(13 例)。共有 10 名肝门型患者接受了不必要的切除手术。修订后的2020年标准有助于诊断肝外病变,而2012年标准中未包括肝外病变。根据2012年标准进行诊断时,需要进行类固醇试验的比例为37.7%,而根据修订后的2020年标准进行诊断时,这一比例降至7.6%。诊断特异性也从2012年标准的58.5%提高到修订后的2020年标准的88.7%:我们对诊断 IgG4-SC 的 2020 年标准的验证有助于避免对孤立 IgG4-SC 患者进行不必要的切除术,IgG4-SC 可分为肝门型和肝外型。2020年标准可提高孤立性IgG4-SC的诊断率,并在纳入影像学检查结果的基础上发现这一难以诊断的实体,减少对类固醇试验的依赖。
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来源期刊
Journal of Hepato‐Biliary‐Pancreatic Sciences
Journal of Hepato‐Biliary‐Pancreatic Sciences GASTROENTEROLOGY & HEPATOLOGY-SURGERY
自引率
10.00%
发文量
178
审稿时长
6-12 weeks
期刊介绍: The Journal of Hepato-Biliary-Pancreatic Sciences (JHBPS) is the leading peer-reviewed journal in the field of hepato-biliary-pancreatic sciences. JHBPS publishes articles dealing with clinical research as well as translational research on all aspects of this field. Coverage includes Original Article, Review Article, Images of Interest, Rapid Communication and an announcement section. Letters to the Editor and comments on the journal’s policies or content are also included. JHBPS welcomes submissions from surgeons, physicians, endoscopists, radiologists, oncologists, and pathologists.
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