Autoimmunpankreatitis – the new kid on the block

Goni, E.; Beyer, G.; Peterhansl, J.; Seidensticker, M.; Schulz, C. und Mayerle, J. (2018): Autoimmunpankreatitis – the new kid on the block. In: Gastroenterologe, Bd. 13, Nr. 6: S. 425-435

Abstract

Autoimmune pancreatitis (AIP) is arare form of chronic pancreatitis which is characterized by diffuse or focal pancreatic enlargement, alymphoplasmacytic infiltrate with astoriform fibrosis and adramatic response to steroid treatment. AIP can be classified into three subtypes: lymphoplasmacytic sclerosing pancreatitis (LPSP;AIP type1), idiopathic duct centric pancreatitis (IDCP;AIP type2), and not otherwise specified (NOS). AIP type1 is the pancreatic manifestation of IgG4-related disease which also involves other organs;AIP type2 is apancreas-specific, IgG4-negative disorder and is associated with inflammatory bowel disease. Epidemiological studies from Japan estimate an incidence of approximately 0.82 per 100,000 inhabitants;in Europe 5-6% of all patients with chronic pancreatitis are diagnosed with AIP. The pathomechanism of AIP remains enigmatic, but amulitfactorial etiology with genetic predisposition and environmental influences has been proposed. Clinically, patients can present with jaundice, epigastric pain, weight loss and new-onset diabetes, thus, raising suspicion for pancreatic malignancy. Acombination of the HISORt (Histology, Imaging, IgG4 Serology, other Organ involvement and Response to steroid therapy) criteria can be used to definitively diagnose AIP in most cases. The greatest challenge is to differentiate AIP from pancreatic cancer and cholangiocarcinoma, since it often presents with apancreatic mass, lymphadenopathy and bile duct obstruction. The prognosis of AIP is usually favorable;in over 90% of cases remission can be induced by steroid treatment.

• Dokumententyp: Zeitschriftenartikel
• Fakultät: Medizin
• Themengebiete: 600 Technik, Medizin, angewandte Wissenschaften > 610 Medizin und Gesundheit
• ISSN: 1861-9681
• Sprache: Deutsch