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Case Reports
. 2015 Sep 7;21(33):9808-16.
doi: 10.3748/wjg.v21.i33.9808.

IgG4-unrelated type 1 autoimmune pancreatitis

Eriko Nakano  1 , Atsushi Kanno  1 , Atsushi Masamune  1 , Naoki Yoshida  1 , Seiji Hongo  1 , Shin Miura  1 , Tetsuya Takikawa  1 , Shin Hamada  1 , Kiyoshi Kume  1 , Kazuhiro Kikuta  1 , Morihisa Hirota  1 , Keisuke Nakayama  1 , Fumiyoshi Fujishima  1 , Tooru Shimosegawa  1
Affiliations
Case Reports

IgG4-unrelated type 1 autoimmune pancreatitis

Eriko Nakano et al. World J Gastroenterol. .

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.

Keywords: IgG4-related disease; Intestinal nephritis; Other organ involvement; Steroid.

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Figures

Figure 1
Figure 1
Findings of fluorodeoxyglucose-positron emission tomography. Positron emission tomography revealed high fluorodeoxyglucose uptake in various organs (uptake is indicated by circles). A: Submandibular glands (SUVmax 5.6) and parotid glands (SUVmax 5.8); B: Pancreas (SUVmax 4.2); C: Bilateral hilar of lymph nodes (SUVmax 7.3) and both kidneys (SUVmax 6.2).
Figure 2
Figure 2
Findings of abdominal computed tomography. A: Localized swelling of the head of the pancreas (short arrows); B: Mild dilation of the upstream main pancreatic duct (long arrow), a thickened common bile duct wall (short arrow), and swelling of the kidneys.
Figure 3
Figure 3
Findings of endoscopic ultrasonography. Endoscopic ultrasonography revealed hypoechoic swelling of the pancreatic head (A: arrowheads), mild dilation of the upstream main pancreatic duct (B: arrowheads), and diffuse thickness of the common bile duct wall (C: arrowheads).
Figure 4
Figure 4
Findings of endoscopic retrograde cholangiopancreatography and intraductal ultrasonography. Endoscopic retrograde cholangiopancreatography revealed an irregular narrowing of the main pancreatic duct (MPD) in the pancreatic head and dilation of the upstream MPD (A); There was a lower bile duct stricture (B); and intraductal ultrasonography showed diffuse thickening of the bile duct wall (C).
Figure 5
Figure 5
Histological findings of pancreas specimens. The histological examination revealed marked lymphoplasmacytic infiltration, storiform fibrosis, and obliterative phlebitis (arrowheads). Immunostaining showed CD38-positive plasma cells and CD163-positive spindle macrophage infiltration. However, no IgG4-positive plasma cells were detected. A: Hematoxylin and eosin staining (magnification × 200 ); B: Elastica-Masson’s staining (magnification × 200); Immunohistochemical staining for CD38 (C), CD163 (D), and IgG4 (E) are also shown at magnification × 100.
Figure 6
Figure 6
Findings of computed tomography after steroid therapy. Computed tomography showed improved the enlargement of the pancreas (indicated arrowheads) and kidneys.
Figure 7
Figure 7
Findings of fluorodeoxyglucose-positron emission tomography after steroid therapy. Fluorodeoxyglucose (FDG)-positron emission tomography showed reduced FDG uptake in various organs.
Figure 8
Figure 8
Histological findings of pancreas and salivary gland specimens. Pancreas and salivary gland tissues exhibited increased CD3-positive T cell infiltration compared with CD20-positive B cell infiltration. A-C: Pancreas (magnification × 100); D-F: salivary gland (magnification × 100); A, D: Hematoxylin and eosin staining; B, C, E, F: Immunohistochemical staining for CD38 (B, E) and CD20 (C, F).
See this image and copyright information in PMC

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