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Table 3 Clinical characteristics of pediatric patients diagnosed with AIP and IFP (3-5).

From: Successful treatment of pediatric IgG4 related systemic disease with mycophenolate mofetil: case report and a review of the pediatric autoimmune pancreatitis literature

  Case report Patient and demo-graphics Signs/
symptoms
Histology Imaging Serology (mg/dl) Other organ systems Response to steroids Treatment Outcome
AIP/likely AIP this report 13yo female fever, headache, joint pain, vomiting, epigastric pain, shortness of breath, weight loss, jaundice EUS-FNA consistent with chronic pancreatitis, duodenal ampulla enlarged, duodenal biopsy with atypical lymphocytic infiltrate focal hypoechoic areas in head, body, and tail of pancreas with enlargement of the head of the pancreas, distal stricture of CBD with biliary dilatation IgG4 226, other autoantibodies negative mediastinal fibrosis, pulmonary nodules, multiple hypodense foci in kidneys, improvement in pain, unable to taper without return of symptoms mycophenolate mofetil resolution of symptoms and abnormalities on imaging, normalization of IgG4
  Fukumori et al. (14) 17yo female severe epigastric and back pain not done US, CT normal, MRCP-MPD only in head of pancreas IgG 2155, IgG4 157, positive antilactoferrin Ab, ANA 1:80 (speckled), other autoantibodies negative none noted resolution of pain, entire MPD visualized on MRCP, disappearance of ALF Ab 30 mg prednisolone, tapered off at 8 months asymptomaticand not currently treated
  Pace et al. (8) 18yo male recurrent acute pancreatitis and cholestasis lymphocytes, macrophages, plasma cells consistent with AIP enlarged pancreatic head on endoscopy IgG4 23, ANA 1:320, other autoantibodies negative none reported resolution of symptoms initially started on prednisolone then tapered off asymptomatic without further treatment
  Blejter et al. (9) 16yo male pruritus and weight loss chronic pancreatitis with interstitial periductal lymphoplasmacytic infiltration and interstitial fibrosis enlarged pancreatic head, dilated biliary tract, no passage of contrast into duodenum on cholangio-graphy IgG4 normal, hypogammaglob-ulinemic, (no specific values given), other autoantibodies negative none reported resolution of symptoms, repeat cholangiogram without biliary dilatation or stricture prednisone 40 mg/kg/day then tapered no recurrence, he requires NPH insulin for diabetes
  Refaat et al. (10) 11yo male nausea, vomiting, dull epigastric pain, anorexia, diarrhea periductal fibrosis, lymphocyte-plasmic parenchymal infiltrate enlarged hypoechoic pancreatic head (US), hypointense surrounding rim (MRI T2-W), diffuse irregular narrowing of main pancreatic duct IgG4 and IgG normal, (no specific values given), other autoantibodies negative none reported not reported not reported not reported
  Gargouri et al. (11) 10yo male severe abdominal pain, biliary vomiting, weight loss not done enlarged pancreas (US), multiple stenoses of Wirsung duct (MRCP), multiple stenoses without intracanalar lacuna and stenosis of retropancrea-tic segment of the bile duct (ERCP) IgG and IgG4 normal (no specific values given), autoantibodies negative none reported resolution of symptoms, normalization of pancreatic size and stenoses of Wirsung duct IV steroids 1 mg/kg/day × 10 days then decreased and discontinued at 7 months asymptomatic, reported 4 years after discontinuation of steroids
  Takase et al. (12) 14yo female severe right upper quadrant pain not done enlargement of pancreas head to tail, homogeneous low-echoic area with some high-echoic spots inside (US), enlargement of the head of the pancreas (CT, MRI), enlarged main pancreatic duct with narrow distal portion (MRCP) IgG 2104 (high) IgG4 54, other autoantibodies negative none reported improvement in symptoms, IgG, MRCP, relapse × 2 (minimum) initial dose IV prednisolone 30 mg/day, multiple tapers and steroid burst, required daily treatment multiple relapses with subsequent imaging changes, no return of elevated IgG
  Bartholomew et al. (13) 10yo male jaundice, intermittent abdominal pain, fatigue, weight loss chronic pancreatitis secondary to lymphoplasmacytic sclerosing pancreatitis pancreatic head mass with likely invasion of portal and superior mesenteric veins (EUS) not reported none reported not given Whipple pancreatico-duodenectomy symptom free at 6 month follow up, requires digestive enzymes
Diagnosed as IFP, examples of case reports that could be consistent with AIP Atkinson et al. (15) 10yo male epigastric pain, jaundice fibrosis enclosing normal acini, with lymphocytes, plasma cells, and leukocytes between acini mass in pancreatic head (laparotomy), obstruction of CBD (IOC) not reported none reported not given cholecysto-duodenostomy symptom free 15 years following surgery
  Elitsur et al. (16) 2yo female abdominal pain fibrous replacement of pancreatic tissue, preservation of Islets of Langerhans, with polymorpho-nuclear cells and plasmalymph-ocytic cells enlarged pancreas with dilatation of proximal bile duct (US, CT), enlarged nodular pancreas with suggestion of retroperitoneal mass (MRI) ANA, anti smooth muscle, anti mitochondrial, antithyroid antibodies all negative, immunoglobulins not reported retroperitoneal mass not given ex-lap for diagnosis, spontaneous resolution of obstruction repeat US revealed normal pancreas and CBD
  Stephen et al. (17) 7yo male jaundice, lethargy, weight loss, prior to jaundice abdominal cramping and vomiting nodular aggregates of lymphocytes and plasma cells, acinar tissue replaced by dense connective tissue enlarged pancreas, head less echogenic than the rest of the pancreas, dilated CBD with tapering at the head of pancreas (US) negative ANA, no other studies reported cholangitis, pericholangitis (similar inflammatory infiltrate) not given Roux-en-Y cholecysto-jejunostomy, incidental appendectomy symptom free
  Keil et al. (18) 14yo male epigastric pain, jaundice severe fibrosis with chronic lymphocytic inflammatory infiltrate enlarged edematous head of pancreas, dilated CBD (US, CT), 2 cm stenosis of CBD (ERCP) "biochemical parameters of inflammatory reactions were normal" none reported not given biliary stenting normal pancreas by US after 12 months of stenting, symptom free 3.5 years following treatment
  1. Abbreviations used: AIP - autoimmune pancreatitis, EUS - endoscopic ultrasound, FNA - fine needle aspirate, CBD - common bile duct, US - ultrasound, CT - computed tomography, MRCP - magnetic resonance cholangiopancreatography,
  2. MPD - main pancreatic duct, Ab - antibody, ANA - antinuclear antibody, ALF - antilactoferrin antibody, MRI - magnetic resonance imaging, T2-W - T2 weighted images, ERCP - endoscopic retrograde cholangiopancreatography, IOC - intraoperative cholangiogram