Autoimmune pancreatitis (patient information)

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Iqra Qamar M.D.[2]

Overview

Autoimmune pancreatitis is a rare form of chronic pancreatitis and is also known as primary inflammatory pancreatitis, lymphoplasmacytic sclerosing pancreatitis, pseudotumorous pancreatitis, chronic pancreatitis with irregular narrowing of the main pancreatic duct, idiopathic chronic pancreatitis, and nonalcoholic duct destructive chronic pancreatitis. Autoimmune pancreatitis may be classified into two types; Type 1 AIP and Type 2 AIP or idiopathic duct-centric pancreatitis. 

What are the Symptoms of Autoimmune pancreatitis?

Patients with autoimmune pancreatitis may do not have a positive history of alcohol abuse. Common symptoms of autoimmune pancreatitis include mild abdominal pain, abdominal mass/pancreatic mass, painless jaundice, weight loss, and diabetes mellitus. Less common symptoms may include symptoms related to other autoimmune diseases such as Sjögren's syndrome, primary sclerosing cholangitis (PSC), inflammatory bowel disease (IBD), and retroperitoneal fibrosis. Autoimmune pancreatitis may have extra-pancreatic involvement such as bile duct, salivary gland, retroperitoneum, kidneys, and orbit depending upon the various stages of autoimmune pancreatitis.

What Causes Autoimmune pancreatitis?

Autoimmune pancreatitis is idiopathic in origin and has no clear etiology. Autoimmune pancreatitis is thought to be due to some autoimmune reaction against pancreas and might be associated with other autoimmune diseases.

Diagnosis

Laboratory findings consistent with the diagnosis of autoimmune pancreatitis may include increased serum IgG4 levels and hypergammaglobulinemia (>2 times the upper limit of normal in most patients), antilactoferrin antibody, anticarbonic anhydrase II antibody, other autoantibodies (ANA), rheumatoid factor (RF), IgG4-positive plasma cells, elevated serum alkaline phosphatase levels (ALP), elevated serum aminotransferases, ESR, and CA19-9.

Endoscopic ultrasound guided fine needle aspiration (EUS-FNA) may be used to obtain histologic specimens from pancreas.

CT scan findings suggestive of autoimmune pancreatitis may include diffusely enlarged pancreas with featureless borders, delayed enhancement with or without a capsule-like rim and rarely calcifications may be seen.

ERCP or MRCP findings in autoimmune pancreatitis may include a narrowed main and dorsal pancreatic duct, diffuse and irregular narrowing of the pancreatic duct (beaded appearance), focal stricture of the pancreatic duct, proximal or distal common bile duct and irregular narrowing of the intrahepatic ducts.

Other diagnostic studies for autoimmune pancreatitis usually include pancreatic biopsy, which may demonstrate extensive fibrosis. Endoscopic ultrasound-guided trucut biopsy (EUS-TCB) is usually avoided due to increased risk of complications and limited diagnostic ability.

Where to find Medical Care for Autoimmune pancreatitis?

Medical care for (disease name) can be found here.

Sources

http://www.nlm.nih.gov/medlineplus/ency/article/000434.htm