Clinical Quiz
Back diagnosis: when pancreatitis is well accompanied
Chiara Rubino 1,2, Mariangela Stinco1,2, Sandra Trapani 2, Elisa Bartolini 1,2
1 Epatologia Pediatrica, Ospedale Pediatrico Meyer, Firenze
2 Pediatria Medica, Ospedale Pediatrico Meyer, Firenze
A 15 year-old patient is conducted to the Emergency Department for asthenia, abdominal pain and mild jaundice. Blood exams show elevated pancreatic enzymes and cholestatic hepatitis. Abdomen ultrasound shows intra- and extra-hepatic biliary dilatation and increased pancreas dimension. During hospitalization, intravenous rehydration and analgesic treatment are started. Cholangio-magnetic resonance confirms the biliary dilatation and pancreatic oedema with mild dilatation of the Wirsung’s duct, in the absence of biliary lithiasis. Among laboratory exams, increased serum IgG4 are detected. In view of the clinical, laboratory and instrumental findings, endoscopic ultrasound-guided pancreatic biopsy is performed and histology is consistent with autoimmune pancreatitis (AIP). Moreover, during hospitalization, the patient undergoes gastrointestinal endoscopy for persistently bloody stools and is diagnosed with ulcerative colitis. Because of the laboratory and instrumental hepatic findings, liver biopsy is performed and pathology is consistent with primary sclerosing cholangitis. The patient starts steroid treatment for the management of autoimmune pancreatitis, ursodeoxycholic acid for liver disease and mesalazine for bowel disease. After the achievement of normal pancreatic findings, azathioprine is added for ulcerative colitis. AIP is rare in paediatric age. The most frequent clinical manifestations are abdominal pain and jaundice, associated with increased pancreatic enzymes and/or altered pancreatic imaging findings. IgG4 are increased in 22% of paediatric cases. Diagnosis should be confirmed by pancreatic biopsy before the start of steroid treatment. As in our case, AIP can be associated with extra-pancreatic diseases, which should be investigated in presence of consistent clinical manifestations or laboratory alterations.