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 - 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.
KW - Autoimmune pancreatitis
KW - Cholangiocarcinoma
KW - IgG4-related disease
KW - IgG4-related sclerosing cholangitis
KW - Isolated IgG4-related sclerosing cholangitis
KW - Primary sclerosing cholangitis
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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 -