Vnitr Lek 2017, 63(4):277-283 | DOI: 10.36290/vnl.2017.056

Relapsing autoimmune pancreatitis type 1: case report

Alena Ondrejková^1,*, Bohuslav Kianička¹, Hana Nechutová¹, Lukáš Hruška², Ivo Novotný³, Miroslav Souček¹

¹ Gastroenterologické oddělení II. interní kliniky LF MU a FN u sv. Anny v Brně

² Klinika zobrazovacích metod LF MU a FN u sv. Anny v Brně

³ Gastroenterologické oddělení MOÚ, Brno

Autoimmune pancreatitis (AIP) is a rare form of chronic pancreatitis, classified into 2 subtypes - AIP type 1 and AIP type 2. We present a case of a 31-years-old female admitted to our institution with upper abdominal pain and obstructive jaundice. Endoscopic retrograde cholangiopancreatoscopy (ERCP) revealed stenosis of intrapancreatic distal bile duct. Diffuse parenchymal enlargement and typical features of AIP were shown by computed tomography (CT) and endoscopic ultrasonography (EUS). The patient's serum IgG4 was elevated at 3.8 g/l (range 0.08-1.4 g/l). She was diagnosed with AIP type 1 and treated with prednisone (initial dose of 30 mg per day, then tapered by 5 mg/day every week). The maintenance dose of 5 mg per day was continued for 6 months. Despite clinical and radiological remission, serum levels of IgG4 remained elevated. The patient experienced disease relapse 25 months after first attack. Moreover, new finding of calcifications occured in pancreas. The relapse was managed with corticosteroids and maintenance immunosupression with azathioprin was started. Literature review on risk factor of relapse, long-term immunosupressive therapy indication and optimal follow-up of AIP type 1 patients are discussed.

Keywords: autoimmune pancreatitis type 1; long-term follow-up; relapse; therapy

Received: February 27, 2017; Accepted: March 18, 2017; Published: April 1, 2017  Show citation