Autoimmune pancreatitis

Medical quality assurance by Dr. Albrecht Nonnenmacher, MD at May 7, 2016
StartDiseasesAutoimmune pancreatitis

Commonly abbreviated as AIP, autoimmune pancreatitis is a medical term that refers to an inflammation of the pancreas (the suffix 'itis' refers to an inflammation) that is believed to be the result of the body's immune system wrongly attacking the pancreas. There are currently two versions of autoimmune pancreatitis, differentiated by the degree and severity of the illness. Only diagnosed as an ailment recently, a number of treatment of options have been shown to effectively treat autoimmune pancreatitis.

Contents

Definition & Facts

AIP or autoimmune pancreatitis is divided into two subtypes. Type 1 AIP is commonly referred to as IgG4-related pancreatitis because the issues with the pancreas are secondary to the IgG4 (sometimes called IgG4-related systemic disease), an autoimmune disease which leads to a complete breakdown of the body's major organs (including the pancreas) via the destruction of connective tissues. Type 2 AIP is when the inflammation is largely contained solely in the pancreas but many people with Type 2 AIP also suffer from digestive ailments like inflammatory bowel disease.

Symptoms & Complaints 

Only recently identified as a separate illness in 1995, a careful analysis must be undertaken in order to differentiate autoimmune pancreatitis from the symptoms of pancreatic cancer, which are very similar. Not everyone with autoimmune pancreatitis has noticeable symptoms. When symptoms do occur, they can be one or more of the following:

Causes

There is much ongoing research about the exact cause of autoimmune pancreatitis. Medical statistics in the United States show an overwhelming predominance of Type 1 AIP with only about one in five people having Type 2 AIP. Known risk factors for developing Type 1 autoimmune pancreatitis include being male, being age 60 or older, IgG4-related diseases, retroperitoneal fibrosis (extra tissue around the stomach and intestines), kidney disease, low thyroid function, and swollen lymph nodes.

Known risk factors for developing Type 2 AIP include being age 40 or older and having digestive illnesses such as inflammatory bowel disease (IBD). Both types of AIP are associated with individuals who have diabetes and stones in their pancreas. Some lines of research have indicated that unrelated problems with the immune system, perhaps triggered by microbial agents, is the primary cause for Type 1 AIP.

The causes of Type 2 AIP are less well-known but it is believed that diabetes or insulin resistance can be a contributing factor to weakening the pancreas and making it more susceptible to attack by the immune system.

Diagnosis & Tests

Most diagnoses of autoimmune pancreatitis begin by identifying the known symptoms and then performing blood tests to gather more information. A high level of IgG4-positive cells can be an important indicator for whether a person has Type 1 autoimmune pancreatitis. The presence of granulocyte epithelial lesions (GELS) in the pancreatic ducts can be an indicator of Type 2 AIP.

CT scans or ultrasounds can be performed to look for pancreatitis. Biopsies of the pancreas may be performed in order to rule out the presence of cancer in the organ or other causes for the inflammation of the pancreas. As the pancreas can become inflamed due a host of other diseases, most diagnoses of autoimmune pancreatitis are made by ruling out other causes in a process known as a differential diagnosis.

Treatment & Therapy

Once autoimmune pancreatitis has been diagnosed as opposed to pancreatic cancer and other illnesses with similar characteristics, treatment can begin. Most forms of current treatment are non-surgical in nature. Steroids are administered for approximately two weeks followed by careful monitoring of the individual to look for reduced discomfort in the abdomen and back, the skin and eyes returning to a normal color, reduced swelling in the pancreas, and blood sugar and pancreatic enzyme levels returning to normal. In most cases of autoimmune pancreatitis, there is a rapid response to the administration of steroids and some doctors use this response to further confirm the initial presence of autoimmune pancreatitis as opposed to other similar illnesses.

Many people with AIP have a chronic form of the disease, meaning it responds well at first to treatment but then flares up again. These individuals may be prescribed a class of drugs called immunosuppressants that work to reduce the amount of damage the immune system is inflicting on the pancreas (and other organs). In some cases, individuals with AIP will also be prescribed supplementary enzymes to replace the ones that a healthy pancreas should be producing.

Prevention & Prophylaxis

Very little is known about how to specifically prevent the onset of autoimmune pancreatitis. Due to the pancreas' function in regulating blood glucose levels and the connection with diabetes, it is believed that all of the standard measures for preventing diabetes and insulin resistance should be taken to prevent further weakening of the pancreas. As Type 1 AIP is related to the inflammation of other organs and glands, it is recommended that a healthy lifestyle and diet be followed in order to prevent problems in those organs.

Currently, there are more than 80 different autoimmune disorders known to medical science. These can be triggered by a host of different reasons, including bacterial infections and viral infections, ingesting toxins, problems with the thyroid gland, the presence of cancer, and blood transfusions and organ transplants. Whenever possible, a healthy lifestyle and diet is recommended as the best way to prevent triggering or developing problems with the immune system.