Diagnosis and management of autoimmune pancreatitis.

• AIP is a heterogeneous disease with two distinct subtypes, now called type 1 and type 2. The proportions of these subtypes vary in their distribution worldwide. • Pancreatic cancer is the leading differential diagnosis for AIP, although AIP can mimic any other major pancreatobiliary disease. • Cross-sectional abdominal imaging CT/MRI should form the cornerstone to the diagnosis of AIP. • Serum IgG4 provides collateral evidence for the diagnosis of AIP and should not be the sole basis for the diagnosis. False-positive elevation in serum IgG4 can be seen in up to 10% of patients with pancreatic cancer. • A steroid trial should be performed only in select situations after ruling out pancreatic cancer and by gastroenterologists experienced in treating AIP. • Disease recurrence can be seen in up to 40% of patients after initial steroid therapy.