I chose pancreatitis for today’s subject because I have heard of it, but I didn’t know there was an autoimmune pancreatitis (AIP). I wanted to know what is the difference between the two and is it something I need to watch for. I have Sjogren’s syndrome which is an autoimmune disorder that AIP is associated with. Ugh!
Pancreatitis is a condition characterized by inflammation of the pancreas.
Pancreatitis has a range of possible causes, including:
- Alcohol use
- Structural problems of the pancreatic and bile ducts
- Some medications like estrogen supplements and some diuretics
- Severe viral or bacterial infection
- Injury to the abdomen
- Elevated triglyceride levels, called hyperlipidemia
- Elevated calcium blood levels, called hypercalcemia
- Genetic causes such as gastric fibrosis
Pancreatitis is categorized as being either acute or chronic.
- Acute pancreatitis generally develops suddenly, and it is usually a short-term (a few days to weeks) illness that typically resolves with appropriate medical management.
- Chronic pancreatitis, which typically develops after multiple episodes of acute pancreatitis, is a long-term condition that can last for months or even several years.
Autoimmune pancreatitis, also called AIP, is a chronic inflammation that is thought to be caused by the body’s immune system attacking the pancreas and that responds to steroid therapy. Two subtypes of AIP are now recognized, type 1 and type 2.
Other differences between type 1 and type 2 AIP are:
- In type 1 AIP, the disease may affect other organs in addition to the pancreas.
- Type 2 AIP affects only the pancreas, although the disease is associated with another autoimmune condition, inflammatory bowel disease.
- Type 1 AIP responds rapidly to treatment with steroids.
- Type 1 AIP is likely to relapse if treatment is discontinued.
6 Autoimmune Disorders Associated with AIP
AIP can occur by itself or in association with other autoimmune diseases such as:
- primary sclerosing cholangitis (PSC) – this is a chronic, or long-term, disease that slowly damages the bile ducts. Bile is a digestive liquid that is made in the liver. It travels through the bile ducts to the gallbladder and the small intestine, where it helps digest fats and fatty vitamins.
- primary biliary cirrhosis – is a progressive disease of the liver caused by a buildup of bile within the liver (cholestasis) that results in damage to the small bile ducts that drain bile from the liver. Over time, this pressure build-up destroys the bile ducts leading to liver cell damage. As the disease progresses and enough liver cells die, cirrhosis and liver failure occur.
- retroperitoneal fibrosis – is a rare condition that’s also known as Ormond’s disease. It occurs when excess fibrous tissue develops in the space behind your stomach and intestine called the retroperitoneal area. Fibrosis is the growth of excess connective tissue, which causes a mass to form.
- rheumatoid arthritis – a chronic progressive disease causing inflammation in the joints and resulting in painful deformity and immobility, especially in the fingers, wrists, feet, and ankles.
- sarcoidosis – a chronic disease of unknown cause characterized by the enlargement of lymph nodes in many parts of the body and the widespread appearance of granulomas derived from the reticuloendothelial system.
- Sjögren’s syndrome – a chronic autoimmune condition characterized by degeneration of the salivary and lachrymal glands, causing dryness of the mouth and eyes.
Symptoms of AIP
- Dark urine.
- Pale stools or stools that float in the toilet.
- Yellow skin and eyes (jaundice)
- Pain in your upper abdomen or middle part of your back.
- Nausea and vomiting.
- Weakness or extreme tiredness.
- Loss of appetite or feelings of fullness.
- Weight loss for no known reason.
How is AIP Diagnosed?
To pinpoint the diagnosis and determine which type of AIP you have, blood and imaging tests are necessary.
Tests may include:
- Imaging tests
- Blood tests
- Endoscopic core biopsy
- Steroid trial
- Biliary stenting. Before starting medication, sometimes doctors will insert a tube to drain the biliary ducts (biliary stenting) in people with symptoms of obstructive jaundice.
- Steroids. Symptoms of autoimmune pancreatitis often improve after a short course of prednisolone or prednisone.
- Immunosuppressants and immunomodulators. About 30 to 50 percent of the time, AIP returns, requiring additional treatment, sometimes long term.
Immunosuppressants and immunomodulators include:
- mercaptopurine (Purinethol, Purixan)
- azathioprine (Imuran, Azasan)
- rituximab (Rituxan).
In general, they have been used in small sample groups and long-term benefits are still being studied.
- Treatment for pancreatic insufficiency. If you have insufficient pancreatic enzymes, you may need supplementary enzymes.
- Treatment for diabetes. If you have diabetes, you will need appropriate treatment.
- Monitoring of other organ involvement. Type 1 AIP often is associated with other organ involvement, including:
- enlarged lymph nodes and salivary glands
- scarring of the bile ducts
- liver inflammation
- and kidney disease.
Although these signs may lessen or disappear completely with steroid therapy, your doctor will continue to monitor you.