| 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 |