Open AccessIgG4-unrelated type 1 autoimmune pancreatitis
Author(s) -
Eriko Nakano,
Atsushi Kanno,
Atsushi Masamune,
Naoki Yoshida,
Seiji Hongō,
Shin Miura,
Tetsuya Takikawa,
Shin Hamada,
Kiyoshi Kaneda,
Kazuhiro Kikuta,
Morihisa Hirota,
Keiichi I. Nakayama,
Fumiyoshi Fujishima,
Tooru Shimosegawa
Publication year - 2015
Publication title -
world journal of gastroenterology
Language(s) - Uncategorized
Resource type - Journals
SCImago Journal Rank - 1.427
H-Index - 155
eISSN - 2219-2840
pISSN - 1007-9327
DOI - 10.3748/wjg.v21.i33.9808
Subject(s) - autoimmune pancreatitis , medicine , endoscopic retrograde cholangiopancreatography , pancreatitis , pathology , pancreas , pancreatic duct , abdominal ultrasonography , lymph , gastroenterology , radiology , computed tomography
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.