TY - JOUR
T1 - Serum IgG4-negative and IgG4-positive type 1 autoimmune pancreatitis present with different clinicopathological features
T2 - An analysis of a nationwide survey in Japan
AU - for the Japan Pancreatitis Study Group for AIP (JPSG-AIP)
AU - Sano, Takanori
AU - Kikuta, Kazuhiro
AU - Takikawa, Tetsuya
AU - Matsumoto, Ryotaro
AU - Okazaki, Kazuichi
AU - Takeyama, Yoshifumi
AU - Masamune, Atsushi
AU - Ikeura, Tsukasa
AU - Itoi, Takao
AU - Ito, Tetsuhide
AU - Inoue, Dai
AU - Irisawa, Atsushi
AU - Ueki, Toshiharu
AU - Uehara, Takeshi
AU - Uchida, Kazushige
AU - Kubota, Kensuke
AU - Kodama, Yuzo
AU - Naitoh, Itaru
AU - Nakazawa, Takahiro
AU - Nishino, Takayoshi
AU - Notohara, Kenji
AU - Hirooka, Yoshiki
AU - Fujimori, Nao
AU - Nakai, Yousuke
AU - Kamisawa, Terumi
AU - Motoya, Masayo
AU - Tomoyuki Ushijima, Kyoko Shimizu
AU - Fukasawa, Mitsuharu
AU - Ueno, Masayuki
AU - Okuwaki, Kosuke
AU - Uza, Norimitsu
AU - Asada, Masanori
AU - Mukai, Tsuyoshi
AU - Kudo, Yasushi
AU - Uetsuki, Kota
AU - Mitoro, Akira
AU - Watanabe, Hiroyuki
AU - Terai, Shuji
AU - Hayashi, Kazunao
AU - Imamura, Yoshiki
AU - Haba, Shin
AU - Hara, Kazuo
AU - Fujisawa, Toshio
AU - Iwasaki, Eisuke
AU - Okumura, Fumihiro
AU - Kawaji, Yuki
AU - Kitano, Masayuki
AU - Nagahama, Masatsugu
AU - Hirano, Atsuyuki
AU - Yamamoto, Satoshi
N1 - Publisher Copyright:
© 2024 IAP and EPC
PY - 2025/2
Y1 - 2025/2
N2 - Background/Objective: Elevated serum IgG4 (sIgG4) is a useful diagnostic marker of type 1 autoimmune pancreatitis (AIP). This study aimed to clarify the clinicopathological characteristics of the type 1 AIP patients without elevated sIgG4 levels. Methods: We analyzed the clinical data of patients registered in a nationwide epidemiological survey in Japan. AIP was diagnosed according to the International Consensus Diagnostic Criteria. Patients with sIgG4 levels ≥135 mg/dl at the diagnosis were classified as sIgG4-positive AIP, and those with sIgG4 levels <135 mg/dl were as sIgG4-negative AIP. Results: A total of 1285 patients with AIP were enrolled in this study; 1128 (87.8 %) had sIgG4-positive AIP and 157 (12.2 %) had sIgG4-negative AIP. Compared to patients with sIgG4-positive AIP, those with sIgG4-negative AIP more frequently experienced inflammatory bowel diseases (3.8 % vs. 0.4 %), and less frequently developed extrapancreatic lesions (53.5 % vs. 72.3 %), including sclerosing cholangitis (30.6 % vs. 40.7 %) and sialadenitis/dacryoadenitis (5.1 % vs. 24.7 %). Histopathological examinations were performed more frequently in patients with sIgG4-negative AIP. The criterion of abundant IgG4-positive plasma cells was less frequently fulfilled by patients with sIgG4-negative AIP (28.0 % vs. 43.1 %). A Kaplan-Meier analysis showed that relapse occurred less frequently in patients with sIgG4-negative AIP (P = 0.006). Results were similar even if the patients with AIP-not otherwise specified (n = 45) were excluded. Conclusions: Patients with sIgG4-negative type 1 AIP and those with sIgG4-positive type 1 AIP present with different clinicopathological features which suggests heterogeneity of patients with type 1 AIP. Low serum IgG4 levels could indicate low disease activity in type 1 AIP.
AB - Background/Objective: Elevated serum IgG4 (sIgG4) is a useful diagnostic marker of type 1 autoimmune pancreatitis (AIP). This study aimed to clarify the clinicopathological characteristics of the type 1 AIP patients without elevated sIgG4 levels. Methods: We analyzed the clinical data of patients registered in a nationwide epidemiological survey in Japan. AIP was diagnosed according to the International Consensus Diagnostic Criteria. Patients with sIgG4 levels ≥135 mg/dl at the diagnosis were classified as sIgG4-positive AIP, and those with sIgG4 levels <135 mg/dl were as sIgG4-negative AIP. Results: A total of 1285 patients with AIP were enrolled in this study; 1128 (87.8 %) had sIgG4-positive AIP and 157 (12.2 %) had sIgG4-negative AIP. Compared to patients with sIgG4-positive AIP, those with sIgG4-negative AIP more frequently experienced inflammatory bowel diseases (3.8 % vs. 0.4 %), and less frequently developed extrapancreatic lesions (53.5 % vs. 72.3 %), including sclerosing cholangitis (30.6 % vs. 40.7 %) and sialadenitis/dacryoadenitis (5.1 % vs. 24.7 %). Histopathological examinations were performed more frequently in patients with sIgG4-negative AIP. The criterion of abundant IgG4-positive plasma cells was less frequently fulfilled by patients with sIgG4-negative AIP (28.0 % vs. 43.1 %). A Kaplan-Meier analysis showed that relapse occurred less frequently in patients with sIgG4-negative AIP (P = 0.006). Results were similar even if the patients with AIP-not otherwise specified (n = 45) were excluded. Conclusions: Patients with sIgG4-negative type 1 AIP and those with sIgG4-positive type 1 AIP present with different clinicopathological features which suggests heterogeneity of patients with type 1 AIP. Low serum IgG4 levels could indicate low disease activity in type 1 AIP.
KW - Autoimmune pancreatitis
KW - Chronic pancreatitis
KW - IgG4-related disease
KW - Inflammatory bowel disease
KW - Pancreatic cancer
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U2 - 10.1016/j.pan.2024.11.018
DO - 10.1016/j.pan.2024.11.018
M3 - Article
AN - SCOPUS:85211178974
SN - 1424-3903
VL - 25
SP - 82
EP - 88
JO - Pancreatology
JF - Pancreatology
IS - 1
ER -