TY - JOUR
T1 - Clinical characteristics of immunoglobulin IgG4-related sclerosing cholangitis
T2 - Comparison of cases with and without autoimmune pancreatitis in a large cohort
AU - Collaborators
AU - Naitoh, Itaru
AU - Kamisawa, Terumi
AU - Tanaka, Atsushi
AU - Nakazawa, Takahiro
AU - Kubota, Kensuke
AU - Takikawa, Hajime
AU - Unno, Michiaki
AU - Masamune, Atsushi
AU - Kawa, Shigeyuki
AU - Nakamura, Seiji
AU - Okazaki, Kazuichi
AU - Furumatsu, Keisuke
AU - Sawai, Shigeaki
AU - Goto, Takuma
AU - Okumura, Toshikatsu
AU - Suzuki, Daisuke
AU - Otsuka, Masayuki
AU - Kobori, Ikuhiro
AU - Tamano, Masaya
AU - Koizumi, Mitsuhito
AU - Hiasa, Yoichi
AU - Kawabe, Naoto
AU - Hirooka, Yoshiki
AU - Yamamoto, Satoshi
AU - Asano, Yukio
AU - Inui, Kazuo
AU - Horiguchi, Akihiko
AU - Watanabe, Hiroyuki
AU - Toya, Daishu
AU - Hatayama, Katsuko
AU - Ueki, Toshiharu
AU - Kinoshita, Norikatsu
AU - Sugimoto, Mitsuru
AU - Ohira, Hiromasa
AU - Mukai, Tsuyoshi
AU - Tomita, Eiichi
AU - Iwata, Keisuke
AU - Shimizu, Shogo
AU - Suetsugu, Jun
AU - Shimizu, Masahito
AU - Tsuji, Keiji
AU - Ishida, Ryoko
AU - Ito, Masanori
AU - Furukawa, Ryutaro
AU - Sakamoto, Naoya
AU - Araki, Masahiro
AU - Tanno, Satoshi
AU - Sakamoto, Yasunari
AU - Ito, Tetsuhide
AU - Takai, Satoshi
N1 - Funding Information:
We deeply appreciate all collaborators in Japan who participated in this nationwide survey, spent valuable time, and registered a plenty of clinical data in the database. The name of collaborators are shown at end of this manuscript. This study was financially supported by the Intractable Hepatobiliary Disease Study Group in Japan, as well as by the Research Committee to establish diagnostic criteria and development of treatment for systemic IgG4-related sclerosing disease, supported by the Research Program of Intractable Disease, both of which were provided by the Ministry of Health, Labor, and Welfare of Japan.
Publisher Copyright:
© 2021 Editrice Gastroenterologica Italiana S.r.l.
PY - 2021/10
Y1 - 2021/10
N2 - Background: The clinical characteristics of IgG4-related sclerosing cholangitis (IgG4-SC) especially without autoimmune pancreatitis (AIP) have not been investigated in a large cohort. Aims: To clarify the clinical characteristics of IgG4-SC and IgG4-SC without AIP. Methods: We retrospectively reviewed imaging, serology, other organ involvement (OOI) and histology of 872 patients with IgG4-SC who participated in a Japanese nationwide survey in 2019, and compared these items between IgG4-SC with and without AIP. Results: AIP was present in 83.7% (730/872) of IgG4-SC. In IgG4-SC, bile duct wall thickening was observed on ultrasound (528/650; 81.2%), computed tomography (375/525; 71.4%) and magnetic resonance imaging or cholangiopancreatography (290/440; 65.9%). An elevated serum IgG4 level (≥ 135 mg/dL) was found in 88.0% (322/366). IgG4-related OOI other than AIP was observed in 25.2% (211/836). The proportion of females was significantly higher in IgG4-SC without AIP (28.9% vs. 20.1%; p = 0.025). Hilar stricture was the most common cholangiographic type in IgG4-SC without AIP (39/107; 36.4%).There were no significant differences between IgG4-SC with and without AIP in the rates of bile duct wall thickening, elevated serum IgG4 level, or IgG4-related OOI. Conclusions: The clinical characteristics of IgG4-SC was similar between IgG4-SC with and without AIP in a large cohort.
AB - Background: The clinical characteristics of IgG4-related sclerosing cholangitis (IgG4-SC) especially without autoimmune pancreatitis (AIP) have not been investigated in a large cohort. Aims: To clarify the clinical characteristics of IgG4-SC and IgG4-SC without AIP. Methods: We retrospectively reviewed imaging, serology, other organ involvement (OOI) and histology of 872 patients with IgG4-SC who participated in a Japanese nationwide survey in 2019, and compared these items between IgG4-SC with and without AIP. Results: AIP was present in 83.7% (730/872) of IgG4-SC. In IgG4-SC, bile duct wall thickening was observed on ultrasound (528/650; 81.2%), computed tomography (375/525; 71.4%) and magnetic resonance imaging or cholangiopancreatography (290/440; 65.9%). An elevated serum IgG4 level (≥ 135 mg/dL) was found in 88.0% (322/366). IgG4-related OOI other than AIP was observed in 25.2% (211/836). The proportion of females was significantly higher in IgG4-SC without AIP (28.9% vs. 20.1%; p = 0.025). Hilar stricture was the most common cholangiographic type in IgG4-SC without AIP (39/107; 36.4%).There were no significant differences between IgG4-SC with and without AIP in the rates of bile duct wall thickening, elevated serum IgG4 level, or IgG4-related OOI. Conclusions: The clinical characteristics of IgG4-SC was similar between IgG4-SC with and without AIP in a large cohort.
UR - http://www.scopus.com/inward/record.url?scp=85101885016&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85101885016&partnerID=8YFLogxK
U2 - 10.1016/j.dld.2021.02.009
DO - 10.1016/j.dld.2021.02.009
M3 - Article
C2 - 33664004
AN - SCOPUS:85101885016
SN - 1590-8658
VL - 53
SP - 1308
EP - 1314
JO - Digestive and Liver Disease
JF - Digestive and Liver Disease
IS - 10
ER -