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Autoimmune pancreatitis. A rare cause of cholestasis
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 b
Department of Gastroenterology, University Hospital of Heraklion, PO Box 1352, Heraklion, Crete, Greece Department of Radiology, University Hospital of Heraklion, PO Box 1352, Heraklion, Crete, Greece
a r t i c l e
i n f o
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 diabetes 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, normal 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, portal venous phase). MRCP (T2WI and projective images) depicted oedematous pancreatic parenchyma (Fig. 2a), segmental pancreatic duct narrowing, common bile duct stenosis and intrahepatic duct dilatation (Fig. 2b). Clinical diagnosis was diffuse autoimmune 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 clinical, 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 findings, 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 pancreatitis. 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
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 b
Department of Gastroenterology, University Hospital of Heraklion, PO Box 1352, Heraklion, Crete, Greece Department of Radiology, University Hospital of Heraklion, PO Box 1352, Heraklion, Crete, Greece
a r t i c l e
i n f o
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 diabetes 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, normal 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, portal venous phase). MRCP (T2WI and projective images) depicted oedematous pancreatic parenchyma (Fig. 2a), segmental pancreatic duct narrowing, common bile duct stenosis and intrahepatic duct dilatation (Fig. 2b). Clinical diagnosis was diffuse autoimmune 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 clinical, 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 findings, 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 pancreatitis. 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