autoimmune pancreatitis. a rare cause of cholestasis

1
Digestive and Liver Disease 43 (2011) e3 Contents lists available at ScienceDirect Digestive and Liver Disease journal homepage: www.elsevier.com/locate/dld Image of the month Autoimmune pancreatitis. A rare cause of cholestasis Mairi Koulentaki a,, Evaggelia Chryssou b , Costantina Coucoutsi a , Ioannis Koutroubakis a a Department of Gastroenterology, University Hospital of Heraklion, PO Box 1352, Heraklion, Crete, Greece b Department of Radiology, University Hospital of Heraklion, PO Box 1352, Heraklion, Crete, Greece article info Article history: Received 8 September 2009 Accepted 6 December 2009 Available online 25 February 2010 A 52-year old woman with a 3-month history of type II dia- betes had recent onset of itching and jaundice. Laboratory tests revealed normal CBC and INR, Glucose 148 mg/dl, AST 194 U/L, ALT 373 U/L, -GT 1425 U/L, ALP 571 U/L, total bilirubin 3.8 mg/dl, nor- mal serum and urine amylase, no recent infection with HAV, HBV, HCV, CMV, and EBV. ANA, anti-DNA, AMA, SMA were negative, IgG 1470 (normal 701–1545 mg/dl), IgG4 1210 (normal 4–230 mg/dl). Multidetector CT showed diffusely enlarged pancreas (Fig. 1a, plain CT), surrounded by a hypo-attenuating rim (Fig. 1b, por- tal venous phase). MRCP (T2WI and projective images) depicted oedematous pancreatic parenchyma (Fig. 2a), segmental pancre- atic duct narrowing, common bile duct stenosis and intrahepatic duct dilatation (Fig. 2b). Clinical diagnosis was diffuse autoim- mune pancreatitis, according to the Asian Diagnostic Criteria [1]. The patient received 40 mg prednisolone (0.6 mg/kg) for 4 weeks followed by a tapering, with immediate resolution of clin- ical, biochemical and immunological parameters. MRCP 4 months post-induction steroid therapy, demonstrated normalization of pancreatic size, CBD and pancreatic duct stricture resolution, and normal appearance of intrahepatic bile ducts (Fig. 2c and d). This excellent response to steroids, with reversion of radiological find- ings, unique to AIP, confirms the diagnosis. A dose of 5 mg/day was maintained for a year; 6 months after steroid discontinuation patient is symptom-free, diabetes is controlled with insulin, IgG4 is normal. Reference [1] Otsuki M, Chung JB, Okazaki K, et al. Asian diagnostic criteria for autoimmune pancreatitis: consensus of the Japan–Korea symposium on autoimmune pancre- atitis. J Gastroenterol 2008;43:403–8. Corresponding author. Tel.: +30 2810 392356; fax: +30 2810 392085. E-mail address: [email protected] (M. Koulentaki). 1590-8658/$36.00 © 2009 Editrice Gastroenterologica Italiana S.r.l. Published by Elsevier Ltd. All rights reserved. doi:10.1016/j.dld.2009.12.002

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Digestive and Liver Disease 43 (2011) e3

Contents lists available at ScienceDirect

Digestive and Liver Disease

journa l homepage: www.e lsev ier .com/ locate /d ld

mage of the month

utoimmune pancreatitis. A rare cause of cholestasis

airi Koulentakia,∗, Evaggelia Chryssoub, Costantina Coucoutsi a, Ioannis Koutroubakisa

Department of Gastroenterology, University Hospital of Heraklion, PO Box 1352, Heraklion, Crete, GreeceDepartment of Radiology, University Hospital of Heraklion, PO Box 1352, Heraklion, Crete, Greece

r t i c l e i n f o

rticle history:

eceived 8 September 2009ccepted 6 December 2009vailable online 25 February 2010

A 52-year old woman with a 3-month history of type II dia-etes had recent onset of itching and jaundice. Laboratory testsevealed normal CBC and INR, Glucose 148 mg/dl, AST 194 U/L, ALT73 U/L, �-GT 1425 U/L, ALP 571 U/L, total bilirubin 3.8 mg/dl, nor-al serum and urine amylase, no recent infection with HAV, HBV,CV, CMV, and EBV. ANA, anti-DNA, AMA, SMA were negative, IgG470 (normal 701–1545 mg/dl), IgG4 1210 (normal 4–230 mg/dl).

Multidetector CT showed diffusely enlarged pancreas (Fig. 1a,lain CT), surrounded by a hypo-attenuating rim (Fig. 1b, por-al venous phase). MRCP (T2WI and projective images) depictededematous pancreatic parenchyma (Fig. 2a), segmental pancre-tic duct narrowing, common bile duct stenosis and intrahepaticuct dilatation (Fig. 2b). Clinical diagnosis was diffuse autoim-une pancreatitis, according to the Asian Diagnostic Criteria

1]. The patient received 40 mg prednisolone (0.6 mg/kg) for 4eeks followed by a tapering, with immediate resolution of clin-

cal, biochemical and immunological parameters. MRCP 4 monthsost-induction steroid therapy, demonstrated normalization ofancreatic size, CBD and pancreatic duct stricture resolution, andormal appearance of intrahepatic bile ducts (Fig. 2c and d). Thisxcellent response to steroids, with reversion of radiological find-ngs, unique to AIP, confirms the diagnosis. A dose of 5 mg/day

as maintained for a year; 6 months after steroid discontinuationatient is symptom-free, diabetes is controlled with insulin, IgG4 isormal.

eference

1] Otsuki M, Chung JB, Okazaki K, et al. Asian diagnostic criteria for autoimmunepancreatitis: consensus of the Japan–Korea symposium on autoimmune pancre-atitis. J Gastroenterol 2008;43:403–8.

∗ Corresponding author. Tel.: +30 2810 392356; fax: +30 2810 392085.E-mail address: [email protected] (M. Koulentaki).

590-8658/$36.00 © 2009 Editrice Gastroenterologica Italiana S.r.l. Published by Elsevieroi:10.1016/j.dld.2009.12.002

Ltd. All rights reserved.