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Autoimmune Pancreatitis - Types, Causes, Symptoms, and Treatment

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Autoimmune pancreatitis is the body's immune-mediated inflammation of the pancreas. The causes, symptoms, and treatments are explained in this article.

Medically reviewed by

Dr. Ghulam Fareed

Published At October 27, 2022
Reviewed AtJanuary 9, 2024

Introduction

Autoimmune pancreatic refers to chronic pancreatic inflammation due to autoimmune manifestations. It was first described by Yoshida et al. in 1995. This type of pancreatitis represents a system autoimmune condition involving other organs, such as the bile duct, lymph nodes, and the retroperitoneum depending on the specific tissue changes observed in a biopsy.

Autoimmune pancreatitis can be named as:

  • Idiopathic duct destructive pancreatitis.

  • Lymphoplasmacytic sclerosing pancreatitis with cholangitis

  • Primary inflammatory pancreatitis.

  • Tumefactive pancreatitis.

  • Non-alcoholic duct destructive chronic pancreatitis.

  • Destructive pancreatitis.

What Is the Difference Between Pancreatitis and Autoimmune Pancreatitis?

Pancreatitis is the inflammation of the pancreas. In comparison, autoimmune pancreatitis is a type of pancreatitis. It is an immunity-mediated condition in which the body's immune system causes inflammation of the pancreas.

What Are the Three Types of Pancreatitis?

The three types of pancreatitis are:

  • Acute Pancreatitis: Inflammation of the pancreas with sudden onset and resolves within a few days. This type of inflammation may not even require antibiotics, pain medications, or intravenous fluid supplies.

  • Chronic Pancreatitis: The onset of symptoms is slow and lasts longer than acute pancreatitis. The patient may need a long hospital stage with pain medication and intravenous hydration.

  • Hereditary: Inflammatory pancreas disorders inherited from one generation to another generation. Diagnosis is made depending on medical and family history.

What Are the Types of Autoimmune Pancreatitis?

  • Type 1 Autoimmune Pancreatitis: This is an IgG4 - related disease which can affect multiple organs along with the pancreas, such as bile ducts, liver, kidneys, salivary glands, and lymph nodes.

  • Type 2 Autoimmune Pancreatitis: This type of autoimmune pancreatitis affects only the pancreas, and in the majority of cases, it is associated with inflammatory bowel disease.

What Are Early Signs of Pancreas Problems?

The early signs of pancreatitis:

  • Nausea.

  • Vomiting.

  • Upper abdominal pain.

  • Tenderness when touching the abdomen.

  • Abdominal pain that radiates to your back.

Symptoms of Autoimmune Pancreatitis:

Autoimmune pancreatitis is known to show no symptoms. However, the signs of pancreatic cancer resemble autoimmune pancreatitis.

These symptoms include:

  • Yellowish discoloration of eyes and skin (jaundice).

  • Pain in the upper abdomen or at the center of the back.

  • Dark-colored urine.

  • Nausea and vomiting.

  • Energy loss and extreme tiredness.

  • Loss of appetite and weight loss.

What Is the Cause of Autoimmune Pancreatitis?

The cause of autoimmune pancreatitis is autoimmune; various immune-mediated mechanisms can trigger the inflammatory response. T-cells mediated immune reaction and shifted to the peripheral blood T- lymphocytes towards T-helper 2 response could also contribute to the recruitment of interleukins and cytokines, inducing inflammation and fibrosis. The IgG4 antibodies are tissue-destructive immunoglobulins in both local and systemic functions.

How to Diagnose Autoimmune Pancreatitis?

The evolution of autoimmune pancreatitis includes imaging of the pancreas and pancreatic duct.

Imaging techniques used are,

  • Magnetic resonance imaging (MRI) shows a diffuse enlargement of the pancreas.

  • Computed Tomography (CT) demonstrates a diffuse or focal enlargement of the pancreas with ill-defined pancreatic clefts. The diffusion makes the pancreas look ‘sausage-shaped.’

  • MRI with cholangiopancreatogram (MRCP) is done to evaluate the size, texture, contrast, and presence of strictures.

  • Other tests, along with imaging, include a comprehensive metabolic panel, complete blood count, and liver and kidney function tests.

Does Pancreatitis Always Show Up on a Computed Tomography?

Computed Tomography is almost 60 % to 95 % sensitive in diagnosing advanced disorders and infections because it can readily detect parenchymal changes associated with inflammation such as pancreatitis.

What Is the Treatment of Autoimmune Pancreatitis?

Administration of corticosteroids leads to improvement in symptoms as well as reducing complications. However, in almost half of the cases, there can be a relapse of autoimmune pancreatitis or failure of steroid treatment. Azathioprine is seen to be effective. Patients with biliary strictures also show a high risk of relapse after withdrawal from steroid therapy.

What to Eat in Cases of Autoimmune Pancreatitis?

To keep the pancreas healthy, it is important to focus on a protein-rich diet along with low animal fat concentrations and is rich in antioxidants. Food items such as beans, lean meats, soups, and dairy alternatives should be consumed. Artificial sweets should be replaced with fruits, as cases of pancreatitis are a higher risk of developing diabetes. Food items such as blueberries, spinach, cherries, and whole grains should be added to the diet to facilitate digestion. Pancreatitis can cause fluid loss. Thus it is important to consume lots of water and fluids.

What Is the Management of Autoimmune Pancreatitis?

1. Dietary Recommendations and changes:

A low-fat diet is often advised to reduce stress on the pancreas. Avoiding fried foods, processed sugars, and excessive red meat consumption can be beneficial. Reduce intake of high-fat foods as they can exacerbate symptoms. Focus on lean proteins, whole grains, fruits, and vegetables. Steer clear of fried foods and processed sugars. Eating smaller, more frequent meals can ease the burden on the pancreas compared to large meals. Staying well-hydrated is important. Water is usually the best choice; however, consult with your healthcare provider if there are specific hydration needs. Limit Alcohol and Caffeine Both can irritate the pancreas. Reducing or eliminating alcohol and moderating caffeine intake might be recommended. Sometimes, deficiencies in certain vitamins (like fat-soluble vitamins A, D, E, and K) can occur due to malabsorption.

2. Importance of Physical Activity and Stress Management:

Regular exercise not only helps maintain a healthy weight but also supports overall well-being. Stress can exacerbate symptoms, so incorporating stress-reduction techniques like meditation, yoga, or mindfulness can be immensely helpful.

3. Avoiding Smoking and Excessive Alcohol:

Both smoking and excessive alcohol can worsen pancreatic health. Steering clear of these substances is crucial in managing AIP.

4. Regular Check-ups and Blood Sugar Monitoring:

Consistent monitoring of blood sugar levels is essential, as pancreatitis can affect insulin production. Routine check-ups with healthcare professionals help track your progress and catch any potential issues early.

5. Support Groups and Resources:

Joining support groups or seeking out resources specific to AIP can provide a sense of community and valuable information. Connecting with others facing similar challenges can be emotionally supportive and educational.

Which Diseases Are Similar to Autoimmune Pancreatitis?

The most similar disease to autoimmune pancreatitis is pancreatic cancer. However, the clinical findings and imaging of the two are very difficult to differentiate. However, the histopathologic examination helps differentiate between the two inflammation and malignancies.

Other diseases which are similar to autoimmune pancreatitis are:

  • Choledocholithiasis: Presence of a minimum of one gallstone in the bile duct. The gallstone can be cholesterol salts, bile pigments, and calcium.

  • Pancreatic Cyst: Overgrowth of pancreatic tissue on its wall. It is benign in nature.

  • Cholangiocarcinoma: Malignancy in the bile duct helps to carry digestive fluid bile.

  • Scarring of Pancreas: Recurrent inflammation and infection can lead to the formation of scars and loss of function.

Conclusion:

Autoimmune pancreatitis is inflammation of the pancreas due to immune-mediated responses. It can be of two types depending on the organs affected. Type 1 affects organs like the bile duct, kidneys, salivary glands, and pancreas. Whereas type 2 affects only the pancreas. Autoimmune pancreatitis symptoms include abdominal pain, pallor, loss of appetite, and weakness. The treatment of choice is steroid therapy in the majority of cases. It is important to focus on a protein-rich diet along with low animal fat concentrations and rich in antioxidants to keep the pancreas healthy. Food items like beans, lean meats, soups, and dairy alternatives should be consumed. Also, blueberries, spinach, cherries, and whole grains should be added to the diet to facilitate digestion.

Frequently Asked Questions

1.

Can Autoimmune Pancreatitis Be Cured?

Autoimmune pancreatitis is a chronic inflammatory disease of the pancreas that is treatable but not considered curable. Treatment options include medications to suppress the immune system and reduce inflammation in the pancreas, such as corticosteroids and immunomodulators. With appropriate treatment, individuals with AIP can attain long-term remission and lead a normal life.

2.

Can Autoimmune Pancreatitis Lead To Death?

In most cases, autoimmune pancreatitis (AIP) is not fatal, but it can cause serious complications if left untreated or not managed properly. Complications may include the development of chronic pancreatitis, pancreatic cancer, or other conditions related to the inflammation of the pancreas. Additionally, some people with AIP may experience complications related to the treatment itself, such as infections or adverse reactions to medication.

3.

What Are the Treatment Options for Autoimmune Pancreatitis?

Autoimmune pancreatitis is typically treated with medications such as corticosteroids and immunomodulators, as well as lifestyle modifications. Individualized treatment is provided according to the specific requirements of each person, and consistent monitoring is essential to manage symptoms and avoid any potential complications.

4.

What Are the Symptoms of Autoimmune Pancreatitis?

The symptoms of autoimmune pancreatitis (AIP) can vary from person to person and similar to those of other pancreatic conditions, such as acute or chronic pancreatitis. Common symptoms of AIP may include abdominal pain, weight loss, jaundice (yellowing of the skin and eyes), nausea and vomiting, and steatorrhea (pale, fatty, and foul-smelling stools). In some cases, AIP may also cause symptoms outside of the pancreas, such as joint pain, skin rash, or dry mouth and eyes. 

5.

In What Situations Should Autoimmune Pancreatitis Be Suspected?

Individuals who exhibit symptoms of chronic pancreatitis and do not have a history of heavy alcohol use or other known causes of pancreatitis may be suspected of having autoimmune pancreatitis (AIP). Additionally, AIP should be considered in individuals who have symptoms of autoimmune diseases, such as joint pain, skin rash, or dry mouth and eyes.

6.

Can Autoimmune Pancreatitis Be Reversed?

Autoimmune pancreatitis is generally not considered to be reversible, as the inflammation can cause permanent damage to the pancreas over time. Treatment is focused on managing symptoms, preventing complications, and reducing inflammation in the pancreas through the use of medications. Early diagnosis and treatment are must to prevent serious complications.

7.

What Kind of Doctor Treats Autoimmune Pancreatitis?

Gastroenterologists, hepatologists, and pancreatologists are the medical specialists who diagnose and treat autoimmune pancreatitis. It is very essential to discuss with a healthcare provider who specializes in gastrointestinal and pancreatic disorders to manage the condition and prevent complications.

8.

Is It Safe to Consume Alcohol if Someone Has Autoimmune Pancreatitis?

It is not safe for individuals with autoimmune pancreatitis to consume alcohol, as it can worsen symptoms, increase the risk of complications, and interfere with the effectiveness of medications used to treat the condition. It is advisable to avoid or limit alcohol consumption and discuss any concerns with a healthcare provider.

9.

Does Autoimmune Pancreatitis Run in Families?

Autoimmune pancreatitis is not considered a hereditary condition, but certain genetic variations may increase the risk of developing it. A family history of autoimmune disorders or AIP may indicate a higher risk, but it does not mean that an individual will develop the condition.

10.

Can a CT Scan Detect the Presence of Autoimmune Pancreatitis?

A CT scan is one of the imaging tests used to diagnose autoimmune pancreatitis (AIP). The scan can reveal signs of inflammation in the pancreas, such as swelling, thickening of the walls, and the presence of a mass. However, a CT scan alone may not be enough to confirm a diagnosis of AIP, and additional tests such as blood tests and a biopsy may be needed.

11.

What Are the Differences Between Autoimmune Pancreatitis and Chronic Pancreatitis?

Autoimmune pancreatitis (AIP) and chronic pancreatitis are two distinct conditions that affect the pancreas. The main differences between these two conditions are:
- Causes: AIP is an autoimmune disorder in which the body's immune system mistakenly attacks the pancreas, whereas chronic pancreatitis is usually caused by long-term alcohol consumption or gallstones.
- Inflammation: In AIP, the inflammation is often more diffuse and involves multiple parts of the pancreas, while chronic pancreatitis typically affects only a portion of the gland.
- Symptoms: The symptoms of AIP may include abdominal pain, weight loss, jaundice, and an enlarged pancreas, whereas chronic pancreatitis can cause persistent abdominal pain, nausea, vomiting, and weight loss.
- Treatment: AIP is usually treated with corticosteroids or immunosuppressive medications, while treatment for chronic pancreatitis often involves lifestyle changes, pain management, and occasionally surgery to remove the damaged portions of the pancreas.

12.

What Does the Term “Focal Autoimmune Pancreatitis” Refer To?

Focal autoimmune pancreatitis (F-AIP) is a subtype of autoimmune pancreatitis (AIP), a rare inflammatory condition that affects the pancreas. In F-AIP, the inflammation is limited to one area or lobe of the pancreas, whereas in the more common diffuse form of AIP, the inflammation is more widespread. The diagnosis of F-AIP is often challenging and requires a combination of clinical, imaging, and histological findings. Treatment typically involves the use of corticosteroids or other immunosuppressive agents.

13.

Is There a Cure for Pancreatitis?

The curability of pancreatitis is determined by the severity of the condition and the root cause. Acute pancreatitis can often be treated and resolved, while chronic pancreatitis is a long-term condition that can cause permanent damage.

14.

What Does the Term “Type 2 Autoimmune Pancreatitis” Refer To?

Type 2 autoimmune pancreatitis refers to a rare type of chronic pancreatitis that is caused by an autoimmune reaction. It is characterized by inflammation and damage to the pancreas, as well as the presence of certain antibodies in the blood. Type 2 autoimmune pancreatitis is often treated with immunosuppressive therapy, which can help to reduce inflammation and prevent further damage to the pancreas.
Dr. Ghulam Fareed
Dr. Ghulam Fareed

Medical Gastroenterology

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pancreatitisautoimmune disorder
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