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Patient with Relapsing Pancreatitis and Cholestasis. Klinik und Poliklinik für Gastroenterologie & Rheumatologie Department für Innere Medizin, Neurologie & Dermatologie Universitätsklinikum Leipzig, AöR. Admission to the Central Emergency Unit August 25 2009. Dieter H., * August 21, 1951
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Patient with Relapsing Pancreatitis and Cholestasis Klinik und Poliklinik für Gastroenterologie & Rheumatologie Department für Innere Medizin, Neurologie & Dermatologie Universitätsklinikum Leipzig, AöR
Admission to the Central Emergency Unit August 25 2009 • Dieter H., * August 21, 1951 • Belt like pain in the upper abdomen since 3 days • Watery stool since 4 days • Weight loss of 3 kg within one week J. Mössner 2010
History • No cases of pancreatitis in the family • Stopped smoking 10 years ago • 1 – 2 l of beer daily until June 2009 • Physical examination • Icterus, abdomen soft, discrete tenderness in upper abdomen, peristalsis rare • ECG normal • Laboratory • Abnormal: ASAT 10,8 µkat/l, ALAT 4,61 µkat/l,gGT 8,92 µkat/l, bilirubin 50,8 µmol/l, CrP 20,5 mg/l • serum electrophoresis: polyclonal hypergammaglobulinemia • Normal: Leukocytes 5,6 gpt/l, lipase J. Mössner 2010
What would you have done? J. Mössner 2010
Abdominal Sonography • Bile ducts dilated, thickened wall of main bile duct and gall bladder • Pancreas: normal size, lobulated, pronounced echos Endosonography • Echos reduced, lobulated pancreas, pancreatic duct not fully visible J. Mössner 2010
Sonography Pancreas enlarged Multiple enhanced echos J. Mössner 2010
Sonography Dilated bile ducts J. Mössner 2010
CT Abdomen • Multiple lymphe node enlargements • No signs of pancreatitis Esophago-Gastro-Duodenoscopy • Macroscopic suspicion of villous atrophy • Celiac disease histopathologically + immunologically verified Colonoscopy • Normal J. Mössner 2010
ERC Dilated bile ducts J. Mössner 2010
Aspiration Cytology from Bile Duct J. Mössner 2010
H31615-09 Proximal Bile Duct J. Mössner 2010
H31615-09 Distal Bile Duct J. Mössner 2010
What would you do? J. Mössner 2010
Hospitalization August 25 – September 2, 2009 ERCP • Successful change of stent placed due to bile duct stenosis J. Mössner 2010
Repeat of HospitalizationSeptember 15 – 22, 2009 J. Mössner 2010
Extended Laboratory Autoantibodies • IgG + IgM (quantitative): normal • IgG4 elevated • 2,31g/l • Carboanhydrase-Ab detectable • ANA 1:160 • Transglutaminase-Ab elevated • IgA > 100 U/ml J. Mössner 2010
Sonographically Guided Pancreas Puncture J. Mössner 2010
H29736-09 Pancreatic Biopsy J. Mössner 2010
H29736-09 Pancreatic Biopsy J. Mössner 2010
H31506-09 Pancreatic Biopsy J. Mössner 2010
H31506-09 Pancreatic Biopsy J. Mössner 2010
Which Diagnosis ? J. Mössner 2010
Sonographically Guided Pancreas Puncture Fibrotic and partly sclerotic parenchyma of pancreas Rarified parts of exocrine and endocrine pancreas Focal proliferation of ducts Lymphoplasmocytic inflammatory infiltration J. Mössner 2010
Normalisation of Enlarged Pancreas after Therapy with Steroids J. Mössner 2010
ERC after Therapy J. Mössner 2010
Relapse of marked bile duct stenosis within the hilus and intrahepatically after rapid cessation of steroid therapy J. Mössner 2010
Ig4-associated AutoimmunePancreatitis & -Cholangitis • IgG4 elevated • In addition celiac disease • Histology of Pancreas • Therapy: • 40 mg Prednisolone / day Kamisawa et al: World J Gastroenterol 2008 J. Mössner 2010
Follow-up of our Patient • Regular control in our ambulance • Tapering of prednisolone until December 2009 • Exchange of stents 10/09, 12/09 and 01/10 • 01/10: Renewal of prednisolone 40 mg/d + azathioprine 50 mg/d J. Mössner 2010
Conclusion • Autoimmune pancreatitis: • rare but possibly underdiagnosed form of pancreatitis • Clarification of dignity of a mass or a stenosis is a must! J. Mössner 2010
