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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