Thus, as already mentioned, type 1 AIP is a systemic disease that can involve the bile ducts, retroperitoneum, lymph nodes, kidneys, and lacrimal and salivary glands, in addition to the pancreas. The involvement of these organs can occur before or contemporaneously with or after pancreatic involvement. The affected organs often share the histological hallmark of type 1 AIP. Thus, patients can present with symptoms indicating other organ involvement, such as dry mouth and dry eyes (a Sjögren-like

syndrome), retroperitoneal fibrosis, orbital pseudotumors, and diffuse or focal lymphadenopathy. Conversely, other organ involvement is not typical of type 2 AIP, although an association with selleck inhibitor inflammatory bowel disease has been reported. Less commonly, AIP can mimic other pancreatic disease in its presentation. For example, patients can present with mild abdominal pain and pancreatic enzyme elevation, suggestive of acute pancreatitis,

or alternatively, pancreatic calcification and steatorrhea selleck might suggest chronic pancreatitis. Rarely, when bile duct involvement precedes pancreatic involvement, the clinical presentation can be similar to that of cholangiocarcinoma. Occasionally, in the post-acute phase, AIP can present with atrophy of the pancreatic parenchyma associated with steatorrhea. In up to 60–70% of cases, diabetes mellitus or impaired fasting glucose is a complication of AIP.11,20,21 Interestingly, glycemic control improves in a subset of AIP patients following the introduction of corticosteroid therapy. Although there are case reports of patients with AIP who also had or subsequently developed pancreatic cancer, there is no firm evidence of a causal link between Fossariinae the two conditions. Pancreatic imaging is the cornerstone to the diagnosis of AIP. Cross-sectional abdominal imaging modalities, such as computed tomography (CT) or magnetic resonance imaging (MRI), are often carried out as part of the initial testing for obstructive jaundice. The presence of

an enlarged, “sausage-shaped pancreas” with featureless borders and rim enhancement is characteristic of AIP.22 Although MRI of the abdomen is comparable to a CT scan, its higher cost and lesser availably limit its usage. On MRI, T1-weighted images of the pancreas are often less intense than T2-weighted images when compared to the liver.23 Ductal changes, such as a long, narrow stricture with no upstream dilatation, are useful clues to the correct diagnosis when present, but they are not always seen on MRI. Focal involvement of the pancreas in AIP can mimic pancreatic cancer. However, a few features of the cross-sectional imaging can distinguish the two. First, the presence of a low-density mass, abrupt cut off the main pancreas duct, and/or atrophy distal to the duct cut off are features that suggest cancer rather than AIP.