Autoimmune Liver Disease - Causes, Types, Symptoms, Diagnosis, Treatment, and Complications

Introduction:

Autoimmune liver disease causes damage and swelling or inflammation of the liver. This happens when the body's immune system (infection-fighting system) attacks its healthy liver. The causes of autoimmune liver disease are unclear, but some factors, like viral infections or certain medications, can trigger or induce the immune system to attack its healthy organs.

The symptoms appear as abdominal pain, yellowish discoloration of the skin and eyes, dark-colored urine, pale color stools, itching, rashes on the skin, joint pain, tiredness, liver enlargement, and absence of menstrual cycle. Autoimmune hepatitis (swelling of the liver) is found with a group of blood tests like liver function tests, complete blood tests, autoimmune liver disease panels, and imaging techniques like ultrasound, CT scan, MRI scan, and biopsy. The treatment of autoimmune hepatitis is done by administering immunity suppressing medications like Prednisolone, Corticosteroids, Azathioprine, and liver transplant in liver failure cases. If there is a delay in finding and treating the disease, the damage to the liver will be severe, leading to cirrhosis and liver failure.

What Is Autoimmune Liver Disease?

The immune system in our body functions as a police force. It attacks infectious agents like bacteria, viruses, or any other agents that cause damage to the body’s healthy cells. The body’s immune system damages its own healthy body cells and tissues in autoimmune disease. This immune system attack on a healthy liver is called autoimmune liver disease. This is a long-term inflammatory disease causing damage to the liver. It does not spread from one person to another person. It occurs in people of all age groups.

What Causes Autoimmune Liver Disease?

The causes of autoimmune liver disease are unclear. However, some factors like getting a viral infection or taking certain medications can spark the immune system to damage its healthy organs.

• Viral Infections: Viral infections like herpes simplex (a viral infection that causes blisters around the mouth) and viral hepatitis A, B, C, and D.

• Medications: Medications like Nitrofurantoin (an antibiotic to treat urinary tract infection), Hydralazine (a medication used to control high blood pressure), and Minocycline (an antibiotic used to treat bacterial infection).

• Diseases: Patients with an already existing autoimmune disease like diabetes mellitus, Graves disease (hyperthyroidism- overproduction of thyroid hormones), celiac disease (a long-term digestive system disease that damages the small intestine), ulcerative colitis (ulcers in the lining of the large intestine and rectum), and thyroiditis (swelling of the thyroid gland and results in increased or decreased production of thyroid hormones) can cause autoimmune liver disease.

What Are the Types of Autoimmune Liver Disease?

• Autoimmune Hepatitis: Autoimmune hepatitis is the inflammation of the liver due to an autoimmune response. Acute hepatitis can be of two types:

  1. Type 1 Acute Hepatitis: This type 1 autoimmune hepatitis is the most common and can occur in anyone. This autoimmune hepatitis occurs in patients who already have an autoimmune disease like celiac disease, rheumatoid arthritis (an autoimmune disease that damages the joints and causes swelling), or ulcerative colitis.

  2. Type 2 Acute Hepatitis: This type of hepatitis is most common in children and young adults. It can also occur in adults.

• Primary Biliary Cholangitis (PBC): Primary biliary cholangitis is a chronic (long-term) disease that affects the bile ducts. The bile duct is tubular in shape that carries bile from the liver and the gallbladder to the small intestine. PBC is an autoimmune disease that injures the bile duct slowly over time and results in permanent damage to the bile duct.

• Primary Sclerosing Cholangitis (PSC): Primary sclerosing cholangitis is a chronic (long-term disease or the disease can occur again) disease of the bile ducts inside and outside the liver. It causes damage to these bile ducts, and the healthy cells are replaced by scar tissue. This hardens the bile duct and causes narrowing of the bile duct, or it is blocked in some cases. This further causes liver dysfunction.

What Are the Symptoms of Autoimmune Hepatitis?

• Joint pain.

• Nausea and vomiting.

• Jaundice (yellowish discoloration of the skin and eyes).

• Dark-colored urine.

• Pale color stools.

• Pain in the stomach region.

• Enlargement or swelling of the liver.

• Ascites (fluid accumulation in the stomach).

• Feeling weak and tired.

• Loss of appetite.

• Weight loss.

• Absence of menstrual periods.

Who Is at Risk for Autoimmune Liver Disease?

• Women.

• Patients with an already existing autoimmune disease like diabetes, ulcerative colitis, rheumatoid arthritis, or thyroiditis.

• Hereditary (the disease runs in the family).

• History of viral infections like herpes simplex virus, hepatitis A, B, C, or D.

How Is Autoimmune Liver Disease Found?

• Complete Blood Count: Complete blood count is done to check the blood parameters like the red blood cells, white blood cells, platelets, hemoglobin, neutrophils, eosinophils, etc., to check for any changes in the normal ranges of these parameters, which indicates an infection.

• Liver Function Tests (LFT): Liver enzymes like alanine transaminase (ALT), aspartate transaminase (AST), alkaline phosphatase (ALP), gamma-glutamyl transferase (GGT), and lactate dehydrogenase (LD) are checked in a liver function test. Proteins like albumin and globulin, bilirubin, and prothrombin tests are done. Any changes in the standard values of these components indicate liver disease.

• Autoimmune Liver Disease Panel: This is a group of blood tests used to screen for autoimmune diseases in the liver. It involves screening certain antibodies. The increase in the levels of these auto-antibodies confirms the autoimmune disease.

  1. Anti-liver Kidney Microsomal Antibodies (LKM): Anti-LKM is a type of auto-antibody that is present in the blood samples of patients with liver disease.

  2. Anti-mitochondrial Antibodies (AMA): AMA is present in blood samples of patients with autoimmune disease. The mitochondria give energy to cells. AMA forms against mitochondria and attacks them in autoimmune diseases.

  3. Anti-nuclear antibodies (ANA): Antibodies are made by our immune system to fight against infections. In autoimmune diseases, anti-nuclear antibodies are formed and attack the antibodies.

  4. Anti-smooth Muscle Antibodies (ASMA): The immune system produces ASMA in autoimmune diseases. They damage the proteins in the smooth muscle and damage the liver and other tissues.

  5. Serum IgG: Immunoglobulin G is the common antibody present in the blood that fights against infection. The high levels of IgG indicate the presence of an autoimmune disease.

• Ultrasound Scan: In ultrasound imaging, high-frequency sound waves are emitted to obtain details of the internal organs to confirm the diagnosis.

• CT Scan: Computerized tomography (CT) obtains images of bony structures and other internal organs by focusing X-ray radiations at multiple angles through computer technology.

• MRI Scan: Magnetic resonance imaging (MRI) uses radiation waves and magnetic waves to get a detailed photo of the internal organs, soft tissues, and surrounding structures for an elaborate diagnosis and treatment plan.

• Liver Biopsy: In a liver biopsy, a tiny portion of the suspected lesion or the organ is removed by forceps and viewed under a microscope to check for pathological changes that help diagnose the condition.

How Is Autoimmune Liver Disease Treated?

Treatment involves suppressing the immune system activity with medications. However, most patients require lifelong treatment.

1. Corticosteroids (Prednisone): It is an immune-suppressing medication and reduces the inflammation or swelling of the affected organ.

2. Azathioprine: This is an immune-suppressing medication that slows down the immune system.

3. Mycophenolate Mofetil: Mycophenolate mofetil medication is given in patients who are not compatible with Azathioprine. Cyclosporine, Sirolimus, and Tacrolimus are other alternate immunosuppressive medications.

4. Liver Transplant: A liver transplant is done when the liver is severely damaged and has cirrhosis.

What Are the Complications of Autoimmune Liver Disease?

If the autoimmune hepatitis is not detected and treated early, it leads to cirrhosis of the liver. Cirrhosis is a severe liver damage disease where the healthy liver cells are replaced by scar tissues. This hardens the liver and leads to liver dysfunction. This can further lead to liver failure and liver cancer. Ascites are also another complication of autoimmune hepatitis.

What Is the Prognosis of Autoimmune Liver Disease?

The prognosis for autoimmune liver disease is good with timely treatment.

How to Prevent Autoimmune Liver Disease?

Autoimmune liver diseases cannot be prevented. It is an autoimmune response, and the causes are unknown.

Conclusion:

Autoimmune liver disease is a serious condition if not detected and treated early. The disease cannot be prevented or predicted since it is an autoimmune response. However, the symptoms can be improved by following a healthy lifestyle, maintaining ideal body weight by exercising regularly, and eating balanced nutritious food. A balanced diet involves having proteins, carbohydrates, fibers, vitamins, and minerals in equal proportions.

Though the disease causes serious health effects, visiting a doctor earliest as soon as any of the symptoms appear can reduce the severity of the disease.