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Table 2 Summary of demographic, radiographic and clinical information from a review of 18 previously published cases of IgG4-Related gastrointestinal disorders

From: Multivisceral IgG4-related disease presenting as recurrent massive gastrointestinal bleeding: a case report and literature review

Study Age, Gender Clinical Symptoms Laboratory CNS Imaging (modality) Biopsy Source Organ Involvement Treatment, Response
Fong et al., 2013 [11] 42, M Obstructive jaundice, pruritus, pale stools, weight loss, tea-colored urine Increased liver enzymes, IgG4 normal Extrahepatic duct presented with a mural thickening (CT), stricture in the middle of bile duct with proximal biliary dilatation (ERCP) Duodenal ampulla Duodenal, liver Marked
79, M Obstructive jaundice, weight loss Not reported Mild dilatation of the biliary tree (CT) Not reported Pancreas, biliary Surgery
Rungsakulkij et al., 2017 [12] 56, M Obstructive jaundice Increased serum IgG4 Bile duct obstruction(CT); stricture of hepatic duct (ERCP) Ampullary Bile duct Surgery
Cai et al., 2014 [13] 57, M Jaundice, upper abdominal Discomfort IgG4 serum normal Mass observed in the hepatic duct (CT), hepatic duct and proximal bile duct dilatation (MRI) Bile duct Bile duct Surgery
El Euch et al., 2017 [14] 70, M Obstructive jaundice, anorexia, abdominal pain, \weight loss Increased liver enzymes, and Serum IgG4 Diffuse pancreatic swelling and strictures of the main pancreatic duct (CT), thickened rim surrounding the pancreatic duct (MRI) Not reported Pancreatic Marked
Miki et al., 2015 [15] 69, M Jaundice, steatorrhea Increased liver enzyme, and serum IgG4 Thickening of the bile duct wall, compressing the right portal vein (CT); bile duct lesions involving the left and right hepatic ducts (ERCP) Bile duct Bile duct Surgery
Sivakumaran et al.., 2014 [16] 51, F Jaundice, weight loss Increased carbohydrate antigen 19–9 Intrahepatic duct dilatation and a hilar stricture (CT), a mass at the portal hepatitis (MRI) Liver liver Surgery, Marked
Rodriguez et al., 2016 [17] 55, F Abdominal pain, weight loss IgG4 and other blood indices were normal A mass in the pancreas(CT), hypermetabolic of the pancreas tail, bone marrow, and spleen, diffuse lymphadenopathy (PET) Bone marrow Pancreas Marked
Kondo et al., 2016 [18] 78, M Bilateral leg edema Not reported Sclerosing cholangitis (MRI) Pleural Pleura, bile duct, pericardium Marked
Yang et al., 2015 [19] 60, M Acid reflux Increased serum IgG4 Multiple masses in the esophagus stomach, and liver (CT) Esophagus, stomach Esophagus, stomach and liver Marked
Miyajima et al., 2017 [20] 50, M Right upper pain, anorexia Leucocytosis Two liver masses (CT) Liver Liver Surgery
Li et al., 2016 [21] 57, F Pruritus Increased liver enzymes and serum IgG4 Dilation of intrahepatic bile duct and lesions occupying the on head of pancreas, anastomotic stenosis (MRCP) Biliary Biliary Surgery
Chen et al., 2016 [22] 58, M Pruritus Increased total bilirubin, direct bilirubin, lipase, and serum IgG4 Stricture of the distal bile duct and dilatation of the pancreatic duct (MRI); pancreatic duct with double duct sign (ERCP) Not reported Pancreas Marked
Shimamura et al., 2015 [23] 74, M Not reported Increased serum IgG4 Low-density lesions, rim-like lesions in the bilateral kidneys (CT) Gastric mass and Kidney Stomach and kidney Marked
Bulanov et al., 2016 [24] 62, F Severe weakness and fatigue Hemoglobin reduction, increased serum IgG4 Chronic ulcerative lesion, thickening of the stomach wall(CT) Gastric mass and regional lymph nodes Stomach, lymph nodes Surgery
Takasumi et al., 2016 [25] 63, F Not reported thrombocytopenia, increased serum IgG4 and IgM Enlargement of the submandibular glands, diffuse enlargement of the pancreas (CT) Bone marrow, liver Liver Marked
Kim et al., 2016 [26] 61, M Weakness, easy fatigability, weight loss Increased liver enzymes and serum IgG4 Type IV hilar cholangiocarcinoma with periductal invasion into underlying hepatic parenchyma (MRI), multiple enlarged lymph nodes in left axillary (PET)   Intrahepatic bile duct Marked
Matsunaga et al., 2014 [27] 72, M Not reported Increased hepatobiliary enzymes, serum IgG, and tumor markers Enhancement in the pancreatic head, stenosis in the bile duct head and dilatation (CT), diffuse pancreatic ductal stenosis (MRI) Duodenal papilla Duodenum Not reported
Van et al., 2017 [28] 26, M Lymphadenopathy, splenomegaly Increased IgG4, reduced T lymphocyte Not reported Pancreas Pancreas Not reported
  1. CT computed tomography, ERCP endoscopic retrograde cholangiography, MRI magnetic resonance imaging, PET positron emission tomography