title: IgG4-unrelated type 1 autoimmune pancreatitis.
abstract: A 50-year-old male was referred to our hospital for the evaluation of hyperproteinemia. Fluorodeoxyglucose positron emission tomography revealed high fluorodeoxyglucose uptake in the pancreas, bilateral lacrimal glands, submandibular glands, parotid glands, bilateral pulmonary hilar lymph nodes, and kidneys. Laboratory data showed an elevation of hepatobiliary enzymes, renal dysfunction, and remarkably high immunoglobulin (Ig) G levels, without elevated serum IgG4. Abdominal computed tomography revealed swelling of the pancreatic head and bilateral kidneys. Endoscopic retrograde cholangiopancreatography showed an irregular narrowing of the main pancreatic duct in the pancreatic head and stricture of the lower common bile duct. Histological examination by endoscopic ultrasonography-guided fine-needle aspiration revealed findings of lymphoplasmacytic sclerosing pancreatitis without IgG4-positive plasma cells. Abnormal laboratory values and the swelling of several organs were improved by the treatment with steroids. The patient was diagnosed as having type 1 autoimmune pancreatitis (AIP) based on the International Consensus Diagnostic Criteria. Therefore, we encountered a case of compatible type 1 AIP without elevated levels of serum IgG4 or IgG4-positive plasma cells. This case suggests that AIP phenotypes are not always associated with IgG4.
authors.affiliation: Eriko Nakano, Atsushi Kanno, Atsushi Masamune, Naoki Yoshida, Seiji Hongo, Shin Miura, Tetsuya Takikawa, Shin Hamada, Kiyoshi Kume, Kazuhiro Kikuta, Morihisa Hirota, Tooru Shimosegawa, Division of Gastroenterology, Tohoku University Graduate School of Medicine, Sendai 980-8574, Japan.
authors.forename: Eriko
authors.initials: E
authors.lastname: Nakano
authors.affiliation: Eriko Nakano, Atsushi Kanno, Atsushi Masamune, Naoki Yoshida, Seiji Hongo, Shin Miura, Tetsuya Takikawa, Shin Hamada, Kiyoshi Kume, Kazuhiro Kikuta, Morihisa Hirota, Tooru Shimosegawa, Division of Gastroenterology, Tohoku University Graduate School of Medicine, Sendai 980-8574, Japan.
authors.forename: Atsushi
authors.initials: A
authors.lastname: Kanno
authors.affiliation: Eriko Nakano, Atsushi Kanno, Atsushi Masamune, Naoki Yoshida, Seiji Hongo, Shin Miura, Tetsuya Takikawa, Shin Hamada, Kiyoshi Kume, Kazuhiro Kikuta, Morihisa Hirota, Tooru Shimosegawa, Division of Gastroenterology, Tohoku University Graduate School of Medicine, Sendai 980-8574, Japan.
authors.forename: Atsushi
authors.initials: A
authors.lastname: Masamune
authors.affiliation: Eriko Nakano, Atsushi Kanno, Atsushi Masamune, Naoki Yoshida, Seiji Hongo, Shin Miura, Tetsuya Takikawa, Shin Hamada, Kiyoshi Kume, Kazuhiro Kikuta, Morihisa Hirota, Tooru Shimosegawa, Division of Gastroenterology, Tohoku University Graduate School of Medicine, Sendai 980-8574, Japan.
authors.forename: Naoki
authors.initials: N
authors.lastname: Yoshida
authors.affiliation: Eriko Nakano, Atsushi Kanno, Atsushi Masamune, Naoki Yoshida, Seiji Hongo, Shin Miura, Tetsuya Takikawa, Shin Hamada, Kiyoshi Kume, Kazuhiro Kikuta, Morihisa Hirota, Tooru Shimosegawa, Division of Gastroenterology, Tohoku University Graduate School of Medicine, Sendai 980-8574, Japan.
authors.forename: Seiji
authors.initials: S
authors.lastname: Hongo
authors.affiliation: Eriko Nakano, Atsushi Kanno, Atsushi Masamune, Naoki Yoshida, Seiji Hongo, Shin Miura, Tetsuya Takikawa, Shin Hamada, Kiyoshi Kume, Kazuhiro Kikuta, Morihisa Hirota, Tooru Shimosegawa, Division of Gastroenterology, Tohoku University Graduate School of Medicine, Sendai 980-8574, Japan.
authors.forename: Shin
authors.initials: S
authors.lastname: Miura
authors.affiliation: Eriko Nakano, Atsushi Kanno, Atsushi Masamune, Naoki Yoshida, Seiji Hongo, Shin Miura, Tetsuya Takikawa, Shin Hamada, Kiyoshi Kume, Kazuhiro Kikuta, Morihisa Hirota, Tooru Shimosegawa, Division of Gastroenterology, Tohoku University Graduate School of Medicine, Sendai 980-8574, Japan.
authors.forename: Tetsuya
authors.initials: T
authors.lastname: Takikawa
authors.affiliation: Eriko Nakano, Atsushi Kanno, Atsushi Masamune, Naoki Yoshida, Seiji Hongo, Shin Miura, Tetsuya Takikawa, Shin Hamada, Kiyoshi Kume, Kazuhiro Kikuta, Morihisa Hirota, Tooru Shimosegawa, Division of Gastroenterology, Tohoku University Graduate School of Medicine, Sendai 980-8574, Japan.
authors.forename: Shin
authors.initials: S
authors.lastname: Hamada
authors.affiliation: Eriko Nakano, Atsushi Kanno, Atsushi Masamune, Naoki Yoshida, Seiji Hongo, Shin Miura, Tetsuya Takikawa, Shin Hamada, Kiyoshi Kume, Kazuhiro Kikuta, Morihisa Hirota, Tooru Shimosegawa, Division of Gastroenterology, Tohoku University Graduate School of Medicine, Sendai 980-8574, Japan.
authors.forename: Kiyoshi
authors.initials: K
authors.lastname: Kume
authors.affiliation: Eriko Nakano, Atsushi Kanno, Atsushi Masamune, Naoki Yoshida, Seiji Hongo, Shin Miura, Tetsuya Takikawa, Shin Hamada, Kiyoshi Kume, Kazuhiro Kikuta, Morihisa Hirota, Tooru Shimosegawa, Division of Gastroenterology, Tohoku University Graduate School of Medicine, Sendai 980-8574, Japan.
authors.forename: Kazuhiro
authors.initials: K
authors.lastname: Kikuta
authors.affiliation: Eriko Nakano, Atsushi Kanno, Atsushi Masamune, Naoki Yoshida, Seiji Hongo, Shin Miura, Tetsuya Takikawa, Shin Hamada, Kiyoshi Kume, Kazuhiro Kikuta, Morihisa Hirota, Tooru Shimosegawa, Division of Gastroenterology, Tohoku University Graduate School of Medicine, Sendai 980-8574, Japan.
authors.forename: Morihisa
authors.initials: M
authors.lastname: Hirota
authors.affiliation: Eriko Nakano, Atsushi Kanno, Atsushi Masamune, Naoki Yoshida, Seiji Hongo, Shin Miura, Tetsuya Takikawa, Shin Hamada, Kiyoshi Kume, Kazuhiro Kikuta, Morihisa Hirota, Tooru Shimosegawa, Division of Gastroenterology, Tohoku University Graduate School of Medicine, Sendai 980-8574, Japan.
authors.forename: Keisuke
authors.initials: K
authors.lastname: Nakayama
authors.affiliation: Eriko Nakano, Atsushi Kanno, Atsushi Masamune, Naoki Yoshida, Seiji Hongo, Shin Miura, Tetsuya Takikawa, Shin Hamada, Kiyoshi Kume, Kazuhiro Kikuta, Morihisa Hirota, Tooru Shimosegawa, Division of Gastroenterology, Tohoku University Graduate School of Medicine, Sendai 980-8574, Japan.
authors.forename: Fumiyoshi
authors.initials: F
authors.lastname: Fujishima
authors.affiliation: Eriko Nakano, Atsushi Kanno, Atsushi Masamune, Naoki Yoshida, Seiji Hongo, Shin Miura, Tetsuya Takikawa, Shin Hamada, Kiyoshi Kume, Kazuhiro Kikuta, Morihisa Hirota, Tooru Shimosegawa, Division of Gastroenterology, Tohoku University Graduate School of Medicine, Sendai 980-8574, Japan.
authors.forename: Tooru
authors.initials: T
authors.lastname: Shimosegawa
mesh_terms: D001327:Autoimmune Diseases / Q000097:blood / Q000175:diagnosis* / Q000276:immunology; D015415:Biomarkers / Q000097:blood; D044963:Biopsy, Fine-Needle; D002760:Cholangiopancreatography, Endoscopic Retrograde; D019160:Endosonography; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood*; D007150:Immunohistochemistry; D008297:Male; D008875:Middle Aged; D010182:Pancreatic Diseases / Q000097:blood / Q000175:diagnosis* / Q000276:immunology; D010641:Phenotype; D049268:Positron-Emission Tomography; D011237:Predictive Value of Tests; D013256:Steroids / Q000627:therapeutic use; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
chemical_list: D015415:Biomarkers; D007074:Immunoglobulin G; D013256:Steroids
keywords: IgG4-related disease; Intestinal nephritis; Other organ involvement; Steroid