Background
Case presentation
Parameter | Admission day | Confirmed day | After treatment 8 days | Follow up 1 month | Follow up 3 months | Follow up 6 months | Follow up 9 months |
---|---|---|---|---|---|---|---|
IgG4 (g/L) | – | 29.20 | 18.40 | 9.86 | 4.74 | 1.87 | 1.70 |
IgG (g/L) | – | 24.5 | 19.4 | – | – | 8.80 | – |
CRP (mg/ L) | 5.46 | – | – | – | 2.62 | 1.32 | – |
TBIL (μmol/L) | 12.1 | 11.7 | 56.4 | 23.4 | 14.1 | 7.7 | – |
DBIL (umol/L) | 6.5 | 5.4 | 44.5 | 16.4 | 6.1 | 3.1 | – |
ALT (IU/L) | 41 | 13 | 315 | 134 | 44 | 29 | – |
AST (IU/L) | 40 | 24 | 167 | 57 | 28 | 26 | – |
ALB (g/L) | 30 | 36.4 | 34.8 | 37.1 | 35.2 | 42.1 | – |
PLT(*109/L) | 80 | 95 | 88 | 106 | 81 | 110 | 140 |
HGB (g/L) | 75 | 61 | 98 | 71 | 122 | 114 | 123 |
Discussion and conclusions
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 |