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Deciphering autoimmune pancreatitis, a great mimicker: case report and review of the literature.

Allaparthi S, Sageer M, Sterling MJ - Case Rep Gastrointest Med (2015)

Bottom Line: EUS revealed a diffusely hypoechoic and rounded pancreatic parenchyma with distal common bile duct (CBD) stricture and dilated proximal CBD and common hepatic duct (CHD).Based on clinical, radiological, and immunological findings, a definitive diagnosis of AIP was made.Patient was started on prednisone 40 mg/day and she clinically responded in 4 weeks.

View Article: PubMed Central - PubMed

Affiliation: Lahey Clinic, Burlington, MA 01805, USA.

ABSTRACT
Background. Autoimmune pancreatitis (AIP) is an atypical chronic inflammatory pancreatic disease that appears to involve autoimmune mechanisms. In recent years, AIP has presented as a new clinical entity with its protean pancreaticobiliary and systemic presentations. Its unique pathology and overlap of clinical and radiological features and absence of serological markers foster the disease's unique position. We report a case of diffuse type 1 autoimmune pancreatitis with obstructive jaundice managed with biliary sphincterotomy, stent placement, and corticosteroids. A 50-year-old Caucasian woman presented to our hospital with epigastric pain, nausea, vomiting, and jaundice. Workup showed elevated liver function tests (LFT) suggestive of obstructive jaundice, MRCP done showed diffusely enlarged abnormal appearing pancreas with loss of normal lobulated contours, and IgG4 antibody level was 765 mg/dL. EUS revealed a diffusely hypoechoic and rounded pancreatic parenchyma with distal common bile duct (CBD) stricture and dilated proximal CBD and common hepatic duct (CHD). ERCP showed tight mid to distal CBD stricture that needed dilatation, sphincterotomy, and placement of stent that led to significant improvement in the symptoms and bilirubin level. Based on clinical, radiological, and immunological findings, a definitive diagnosis of AIP was made. Patient was started on prednisone 40 mg/day and she clinically responded in 4 weeks.

No MeSH data available.


Related in: MedlinePlus

ERCP arrows showing CBD and MPD strictures pre stent insertion (a) and (b); post stent insertion (c) and (d) (clockwise).
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fig3: ERCP arrows showing CBD and MPD strictures pre stent insertion (a) and (b); post stent insertion (c) and (d) (clockwise).

Mentions: A 50-year-old Caucasian woman presented to our hospital with 2-day duration of epigastric pain, nausea, vomiting, and jaundice. Her physical examination was unremarkable except for scleral icterus. An abdominal examination revealed epigastric tenderness without rebound. Laboratory investigations revealed hemoglobin 12.9 g/dL, white blood cell count 9.6/μL, serum lipase 109 U/L, serum amylase 10 U/L, and total bilirubin 10.6 mg/dL (direct and indirect fractions 8 mg/dL and 2.6 mg/dL, resp.). Her liver enzymes were elevated (aspartate aminotransferase 110 U/L, alanine aminotransferase 131 U/L, and alkaline phosphatase 389 U/L). Tests for hepatitis A, B, and C are negative and found to have elevated immunoglobulin IgG Ab level of 765 mg/dl. Given these lab findings and clinical presentation, MRCP was further ordered which showed diffusely enlarged abnormal appearing pancreas with loss of normal lobulated contours. The pancreatic parenchyma is diffusely hypoenhancing, with focal hypoenhancement within pancreatic head, distal body, and tail (Figure 1). In view of her clinical presentation with worsening jaundice and MRCP findings, an endoscopic ultrasound (EUS) was done that revealed a diffusely hypoechoic and rounded pancreatic parenchyma with distal common bile duct (CBD) stricture and dilated proximal CBD and common hepatic duct (CHD) (Figure 2). Endoscopic retrograde cholangiopancreatography (ERCP) showed tight mid to distal CBD stricture that needed dilatation, sphincterotomy, and placement of stent that significantly improved her clinical symptoms and bilirubin level (Figure 3). She was started on tapering dose of prednisone 40 mg/day and 4 weeks after treatment she improved clinically and radiologically. Our ability to recognize AIP and differentiate it from pancreatic adenocarcinoma is aided by the use of international consensus criteria.


Deciphering autoimmune pancreatitis, a great mimicker: case report and review of the literature.

Allaparthi S, Sageer M, Sterling MJ - Case Rep Gastrointest Med (2015)

ERCP arrows showing CBD and MPD strictures pre stent insertion (a) and (b); post stent insertion (c) and (d) (clockwise).
© Copyright Policy - open-access
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC4326036&req=5

fig3: ERCP arrows showing CBD and MPD strictures pre stent insertion (a) and (b); post stent insertion (c) and (d) (clockwise).
Mentions: A 50-year-old Caucasian woman presented to our hospital with 2-day duration of epigastric pain, nausea, vomiting, and jaundice. Her physical examination was unremarkable except for scleral icterus. An abdominal examination revealed epigastric tenderness without rebound. Laboratory investigations revealed hemoglobin 12.9 g/dL, white blood cell count 9.6/μL, serum lipase 109 U/L, serum amylase 10 U/L, and total bilirubin 10.6 mg/dL (direct and indirect fractions 8 mg/dL and 2.6 mg/dL, resp.). Her liver enzymes were elevated (aspartate aminotransferase 110 U/L, alanine aminotransferase 131 U/L, and alkaline phosphatase 389 U/L). Tests for hepatitis A, B, and C are negative and found to have elevated immunoglobulin IgG Ab level of 765 mg/dl. Given these lab findings and clinical presentation, MRCP was further ordered which showed diffusely enlarged abnormal appearing pancreas with loss of normal lobulated contours. The pancreatic parenchyma is diffusely hypoenhancing, with focal hypoenhancement within pancreatic head, distal body, and tail (Figure 1). In view of her clinical presentation with worsening jaundice and MRCP findings, an endoscopic ultrasound (EUS) was done that revealed a diffusely hypoechoic and rounded pancreatic parenchyma with distal common bile duct (CBD) stricture and dilated proximal CBD and common hepatic duct (CHD) (Figure 2). Endoscopic retrograde cholangiopancreatography (ERCP) showed tight mid to distal CBD stricture that needed dilatation, sphincterotomy, and placement of stent that significantly improved her clinical symptoms and bilirubin level (Figure 3). She was started on tapering dose of prednisone 40 mg/day and 4 weeks after treatment she improved clinically and radiologically. Our ability to recognize AIP and differentiate it from pancreatic adenocarcinoma is aided by the use of international consensus criteria.

Bottom Line: EUS revealed a diffusely hypoechoic and rounded pancreatic parenchyma with distal common bile duct (CBD) stricture and dilated proximal CBD and common hepatic duct (CHD).Based on clinical, radiological, and immunological findings, a definitive diagnosis of AIP was made.Patient was started on prednisone 40 mg/day and she clinically responded in 4 weeks.

View Article: PubMed Central - PubMed

Affiliation: Lahey Clinic, Burlington, MA 01805, USA.

ABSTRACT
Background. Autoimmune pancreatitis (AIP) is an atypical chronic inflammatory pancreatic disease that appears to involve autoimmune mechanisms. In recent years, AIP has presented as a new clinical entity with its protean pancreaticobiliary and systemic presentations. Its unique pathology and overlap of clinical and radiological features and absence of serological markers foster the disease's unique position. We report a case of diffuse type 1 autoimmune pancreatitis with obstructive jaundice managed with biliary sphincterotomy, stent placement, and corticosteroids. A 50-year-old Caucasian woman presented to our hospital with epigastric pain, nausea, vomiting, and jaundice. Workup showed elevated liver function tests (LFT) suggestive of obstructive jaundice, MRCP done showed diffusely enlarged abnormal appearing pancreas with loss of normal lobulated contours, and IgG4 antibody level was 765 mg/dL. EUS revealed a diffusely hypoechoic and rounded pancreatic parenchyma with distal common bile duct (CBD) stricture and dilated proximal CBD and common hepatic duct (CHD). ERCP showed tight mid to distal CBD stricture that needed dilatation, sphincterotomy, and placement of stent that led to significant improvement in the symptoms and bilirubin level. Based on clinical, radiological, and immunological findings, a definitive diagnosis of AIP was made. Patient was started on prednisone 40 mg/day and she clinically responded in 4 weeks.

No MeSH data available.


Related in: MedlinePlus