Existing criteria are geared towards the diagnosis of type 1 AIP. At present, pancreatic histology is a requirement for the definitive diagnosis of type 2 AIP. AIP can mimic most other pancreatic diseases in its presentation, but

DNA-PK inhibitor in clinical practice, it often has to be differentiated from pancreatic cancer. There are established criteria and algorithms not only to diagnose AIP, but also to differentiate it from pancreatic cancer. The utility of these algorithms and the approach to management are discussed here. Autoimmune pancreatitis (AIP) is a rare but distinct form of chronic pancreatitis. Although the first report of an autoimmune process affecting the pancreas can be attributed to the French group led by Henri Sarles, the term “AIP” was not coined until 1995.1–3 Most early case reports originated in Japan. A critical milestone was reached when Hamano et al. in 2001 described the association between serum immunoglobulin G (IgG)4 and AIP.4 To this day, this has proven to be the most useful serum marker for diagnosing AIP. In 2004, Kamisawa et al. showed that that there is an intense IgG4-positive cell MLN0128 infiltration, not only in the pancreas, but also in the other organs affected by AIP. Thus, the term “IgG4-associated systemic diseases” was coined.5 Over the years, various other names

have been used to describe AIP, such as lymphoplasmacytic sclerosing pancreatitis (LPSP), idiopathic duct destructive pancreatitis, medchemexpress and granulocyte epithelial

lesion (GEL)-positive pancreatitis. The reason for such a plethora of terminology is partly due to the fact that AIP is a heterogenous disease. Observations from Asia differ from those from Europe and the US with regards to clinical presentation and histology. Specifically, reports from Asia predominantly described a disease affecting elderly males, with pancreatic histology showing a lymphoplasmacytic infiltrate. Reports from Europe described a disease which affected both sexes equally, and a pancreatic histology showing a neutrophilic infiltrate called GEL. These differences delayed the formulation of a consensus definition for AIP. This issue was recently addressed during an international consensus meeting for AIP in 2011 under the auspices of the Autoimmune Pancreatitis International Study Group.6 This group gathered leading AIP researchers from around the world, and among other things, the need for uniformity in nomenclature used to describe AIP was addressed. It was agreed upon that LPSP be called type 1 AIP, and GEL-associated AIP be called type 2 AIP. In this review, we will follow this nomenclature, and unless otherwise specified, the generic term “AIP” refers to type 1 AIP. There are numerous diagnostic criteria that can aid the clinician in establishing the diagnosis of AIP. More recently, algorithms for differentiating AIP from pancreatic cancer have been published.