Autoimmune liver disease

    Last updated date: 13-Mar-2023

    Originally Written in English

    Autoimmune Liver Disease

    Autoimmune Liver Disease

    Autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC), and primary sclerosing cholangitis (PSC) are the three main types of autoimmune liver disease. All three of these conditions are well-defined and are diagnosed using a combination of clinical, serologic, and liver pathological findings. Even though these conditions are regarded as autoimmune in nature, their causes and potential environmental triggers are yet unknown. the destruction of small intrahepatic bile ducts and canals of Hering in PBC, the periductal fibrosis and inflammation involving larger bile ducts with variable small duct damage in PSC, and the chronic hepatitis pattern of injury with predominant plasma cells in AIH are the distinctive morphologic patterns of injury. The detection of antimitochondrial antibodies in PBC, anti-nuclear, anti-smooth muscle, and anti-LKM antibodies in AIH, and pANCA in PSC are among the serological results. Even while the majority of autoimmune liver diseases fall clearly into one of these three groups, overlap syndromes, especially AIH with PBC or PSC, can account for up to 10% of cases. Variant syndromes, like antimitochondrial antibody-negative PBC, also happen. Rare are sequential syndromes that change the autoimmune liver disease from one type to another.


    Autoimmune Disease of Liver

    Autoimmune Disease of Liver

    Autoimmune liver disease is one in which the liver is attacked by the immune system. These illnesses can cause cirrhosis, which in turn can cause liver failure.

    • Primary biliary cholangitis (PBC). The small bile channels in the liver become harmed and inflamed in primary biliary cholangitis which is a well-known chronic liver disease.
    • Primary sclerosing cholangitis (PSC). A persistent condition is primary sclerosing cholangitis. The bile ducts both inside and outside of the liver swell, scar, and ultimately narrow or get blocked. Bile accumulating in the body damages the liver.
    • Autoimmune hepatitis. A chronic liver illness called autoimmune hepatitis occurs when the immune system destroys the liver. Untreated autoimmune hepatitis can cause cirrhosis and liver failure.


    Primary Biliary Cirrhosis

    Primary Biliary Cirrhosis

    Primary biliary cirrhosis, also known as primary biliary cholangitis (PBC), is a condition that impairs the liver's capacity to function. Because it is chronic, it lasts a long period or recurs frequently. The bile ducts of PBC patients suffer injury, inflammation, and eventually irreversible impairment. Small tubes in the liver called bile ducts transport bile, a chemical required for food digestion, to other areas of the digestive system. Bile accumulates in the liver when the bile ducts are dysfunctional. This accumulation may result in liver scarring (cirrhosis). The liver fails to perform correctly as a result of cirrhosis. Since PBC is progressive, it deteriorates with time. Cirrhosis can result in liver failure and possibly death if it is not addressed.


    Primary Sclerosing Cholangitis

    Primary Sclerosing Cholangitis

    A very uncommon condition that affects the bile ducts is called primary sclerosing cholangitis (PSC). Scarring is what the word sclerosing signifies. Your bile ducts scar when you have PSC. They gradually close up until bile builds up inside your liver and begins to harm it.

    You require bile, a vital digestive liquid, to break down lipids. A, D, E, and K, which are fat-soluble vitamins, also require it to be absorbed. Your liver produces bile, which is then transported by ducts to your gallbladder and small intestine. PSC harms your liver's bile ducts both internally and externally.


    Autoimmune Hepatitis

    The liver is a sizable organ that is located on the right side of your abdomen, up under your ribcage. It produces bile to aid with food digestion, filters waste from your body, and stores sugar that your body utilizes as fuel. The immune system of your body destroys the liver cells, resulting in autoimmune hepatitis. As a result, the liver is damaged and swells up. It is a chronic or long-term inflammatory liver disease that can strike anyone at any age, affects women more than males, and is frequently associated with other illnesses where the body attacks itself (autoimmune disorders).


    Autoimmune Liver Disease Causes

    Autoimmune Liver Disease Causes


    Primary Biliary Cholangitis Causes

    PBC has an unknown exact cause, as per physicians. According to some specialists, it could be caused by an immune system issue where the body's healthy cells are being attacked. Doctors think the immune system attacks the bile ducts in PBC.

    Some PBC patients also have other autoimmune disorders, though there is no known reason. These diseases include celiac disease, thyroid disorders, Raynaud's disease, autoimmune hepatitis, and scleroderma. In people with PBC, recurrent urinary tract infections are also common. Additionally, PBC may be brought on by infections, smoking, and exposure to specific chemicals. Additionally, PBC seems to affect family members more frequently. The risk for the other family members increases if one family member has PBC.


    Primary Sclerosing Cholangitis Causes

    Nobody is exactly sure what causes PSC. At any age, it can begin. At around the age of 30 to 40, medical professionals frequently make the diagnosis. Men are more likely to have PSC. Among the potential causes are:

    • Genes. Your risk rises if you have family members who suffer from PSC.
    • Germs. PSC may be triggered by viruses and bacteria.
    • Inflammatory bowel disease. Inflammatory bowel disease, mainly ulcerative colitis, affects many persons with PSC.
    • Immune system issues. Your body's defense mechanism is called the immune system. PSC might be affected by an abnormal immune system.


    Autoimmune Hepatitis Causes

    Although the exact origin of autoimmune hepatitis is unknown, those who also have other autoimmune diseases, such as:

    • Thyroiditis
    • Grave's disease
    • Diabetes type 1
    • Hemolytic anemia
    • Immune thrombocytopenia
    • Celiac disease
    • Ulcerative colitis
    • Rarely, some drugs may cause autoimmune hepatitis.


    Autoimmune Liver Disease Symptoms

    Autoimmune Liver Disease


    Primary Biliary Cholangitis Symptoms

    Early on, PBC affects many people without showing any symptoms. When a patient is examined by a doctor for another condition, they can realize they have the condition. Affected persons may initially experience symptoms:

    • Tiredness (fatigue)
    • Skin itch (pruritus)
    • Belly pain
    • Skin turning darker
    • Yellow or white spots under the eyes or the skin
    • Dry mouth and eyes
    • Joint and muscle aches

    Additional signs and symptoms of PBC in later stages include:

    • Whites of the eyes and skin become yellow (jaundice)
    • Swelling in the ankles, feet, and legs (edema)
    • Abdominal swelling as a result of fluid retention (ascites)
    • Internal bleeding from enlarged veins in the upper stomach and lower esophagus (varices)
    • Nausea
    • Loss of weight
    • Urine darkening


    Primary Sclerosing Cholangitis Symptoms 

    PSC symptoms appear gradually. For many years, PSC may be present in you without any symptoms. After they start, symptoms could come and go. But with time, they frequently develop worse. A type of liver scarring known as cirrhosis may develop over time as a result of bile that builds up in your liver. As a result, the liver fails. PSC symptoms include:

    • Skin itch.
    • Extreme tiredness (fatigue).
    • Belly ache.
    • Jaundice is a yellowing of the skin and eyes.
    • Bile duct infection-related chills and fever.


    Autoimmune Hepatitis Symptoms

    Symptoms can differ from person to person. Some of the most obvious symptoms could be:

    • Extreme tiredness (fatigue)
    • Eyes and skin that have become yellow (jaundice)
    • Abdomen (tummy) pain
    • Joint discomfort or swelling
    • Mild flu-like symptoms
    • Huge liver and spleen cause the abdomen to be large.
    • Spider-like skin blood vessels 
    • Itching

    Other symptoms of autoimmune hepatitis include:

    • Dark fecal material
    • Gray or white stools
    • Reduced appetite
    • Fluid retention in the abdomen (ascites)
    • Confusion
    • Rectal hemorrhage or bloody vomiting

    The symptoms of autoimmune hepatitis can resemble those of other illnesses. For a diagnosis, consult your healthcare provider at all times.


    Autoimmune Liver Disease Diagnosis

    Autoimmune Liver Diagnosis


    Primary Biliary Cholangitis Diagnosis

    A doctor will inquire about your medical and family history, perform a physical exam, order blood tests, and perform other tests to diagnose PBC. Anti-mitochondrial antibody (AMA) is a specific chemical that doctors look for in the blood using a blood test. Almost invariably, the presence of this chemical indicates PBC. Patients with PBC may have other blood test results that show higher than normal levels of liver enzymes, cholesterol, and triglycerides. Additionally, liver-specific blood testing will be performed. If you do have PBC, your physician might also perform an ultrasound examination to obtain images of your liver and bile ducts. Doctors can see any other problematic liver conditions using ultrasound. To confirm the diagnosis, a doctor will occasionally do a liver biopsy. In this test, your doctor uses a tiny needle to take a little piece of liver from your body to examine it under a microscope.


    Primary Sclerosing Cholangitis Diagnosis

    When the results of a regular blood test reveal abnormal liver enzymes, your doctor can suspect PSC. When the liver is damaged, these enzymes rise. The diagnosis will probably be confirmed by a bile duct imaging study. Testing might involve:

    • Blood test. These may be used to identify bile duct disorders. They can assess vitamin deficiencies as well.
    • Endoscopic retrograde cholangiopancreatography (ERCP). During this procedure, your doctor uses an endoscope to introduce dye into your bile ducts. A small camera and light are located at one end of this long, thin tube. Your mouth is used to insert the scope, which is then guided into your small intestine. X-ray images are taken by your healthcare provider after the dye injection. In the course of this procedure, the doctor might open obstructed bile ducts.
    • Percutaneous transhepatic cholangiography. In this procedure, a lengthy needle is put through your skin and dye is injected into your bile ducts. Your doctor injects the dye and then performs X-rays.
    • Magnetic resonance cholangiopancreatography (MRCP). A computer and radio waves are used in this study to create images of your bile ducts. If your doctor or other healthcare professional uses dye, they will inject it into a blood vessel. The primary test for identifying PSC is this one.
    • Ultrasound. In these tests, ultrasound waves are used to provide images of the gallbladder, liver, bile ducts, and drainage system.
    • Liver biopsy. The liver tissue is collected for this test. Then it is examined under a microscope. Sometimes it's necessary to check for other disorders that are related.


    Autoimmune Hepatitis Diagnosis

    Your doctor will examine you physically and review your medical history. The following are some lab blood tests used to identify autoimmune hepatitis:

    • Liver function tests. These examine your liver for inflammation or injury.
    • Complete blood count. Examine the number and type of cells in your blood.
    • Coagulation profile. The effectiveness of the clotting proteins is evaluated during this test.
    • Electrolytes panel. Determines whether there is an electrolyte imbalance.
    • Autoimmune antibodies. The purpose of these tests is to determine whether you have autoimmune hepatitis or another liver illness with comparable symptoms.
    • Further liver testing.  These are performed to look for further potential liver diseases.

    You might also undergo imaging tests like:

    • CT scan. Compared to a regular X-ray, this is more accurate. Any aspect of the body, including the bones, muscles, fat, and organs, can be demonstrated in great detail. It creates horizontal images (commonly referred to as slices) of the body using both X-rays and computer technologies.
    • MRI. This examination captures precise images of the internal organs and body parts. It makes use of radio wave energy pulses and a magnetic field. Your vein may be shot or injected with a dye. The dye makes the liver and other abdominal organs easier to see on the scan.
    • Ultrasound. High-frequency sound waves are used in this to visualize the organs. Additionally, it can evaluate blood flow in blood vessels.
    • Liver biopsy. A needle is used to remove small tissue samples from your liver. To determine the form of liver condition you have, these samples are examined under a microscope.


    Autoimmune Liver Disease Treatment

    Primary Biliary Cholangitis


    Primary Biliary Cholangitis Treatment

    PBC has no known treatment options. To control symptoms and halt the course of PBC, doctors prescribe medication. Ursodiol aids in the liver's bile removal. In many instances, this medication enhances liver function and stops the progression of liver disease. If ursodeoxycholic acid (UDCA) is ineffective or intolerable, obeticholic acid is taken alone or in combination with it. This treatment promotes bile flow out of the liver while reducing bile production.

    Depending on the individual circumstances of each patient and the presence of additional medical issues, various pharmacological options may be available. If liver function declines despite medical intervention, a liver transplant may be required. Diphenhydramine (available over-the-counter) or the prescription medications hydroxyzine or cholestyramine may be administered to treat the common issue of intense itching.


    Primary Sclerosing Cholangitis Treatment

    No recognized medication can stop or reduce PSC. Your healthcare practitioner can treat your symptoms by prescribing anti-itch and anti-infection medications. When your liver's ducts get obstructed, the doctor may also be able to open them. You can also require vitamin supplements. PSC advances slowly. But after ten to fifteen years, it might result in liver failure. The majority of patients with liver failure benefit from liver transplant surgery.

    Osteoporosis, or brittle bones, is another fairly typical PSC symptom. Your bone density will need to be monitored. Monitoring vitamin levels will also be necessary. You should undergo a colonoscopy once a year if you suffer from inflammatory bowel disease (IBD).

    When you are diagnosed with PSC, a colonoscopy should be performed to check for colitis even if you do not have IBD. The bile duct, liver, gallbladder, and colon malignancies are also possible complications for people with PSC. You can talk with your healthcare practitioner about screening tests.


    Autoimmune Hepatitis Treatment

    Early diagnosis of autoimmune hepatitis is crucial for successful treatment. Treatment aims to manage the illness and lessen or eliminate any symptoms (be in remission).

    Corticosteroids and immune system suppressors are medications that are used to help suppress or slow down your overactive immune system. Additionally, they prevent the body from targeting your liver.

    The time it takes for the disease to enter remission after you begin treatment might range from six months to several years. Some people can stop taking their medications, but the disease frequently returns. For the rest of your life, you might require therapy periodically. If a person has experienced numerous relapses or if their disease is severe, they may need to continue receiving therapy. Autoimmune hepatitis may occasionally go away on its own without the need for treatment. However, autoimmune hepatitis is typically a chronic condition.

    It may result in liver scarring (cirrhosis). The liver can sustain damage that is so severe that it stops functioning. Doctors refer to this as liver failure. If your liver is failing, a transplant may be necessary. Make sure to consult your doctor about any recommended vaccinations. Among these are vaccinations against viruses that can harm the liver.


    Autoimmune Liver Disease Life Expectancy

    Without treatment, between 40% and 50% of patients with serious illnesses will pass away between six months to five years. Steroid therapy has significantly changed the disease's course. The majority of patients benefit from therapy, and the 10-year survival rate ranges between 83% and 94%.



    The three main autoimmune liver diseases (AILD) are primary biliary cirrhosis (PBC), primary sclerosing cholangitis (PSC), and autoimmune hepatitis (AIH). PBC, PSC, and AIH are all complex illnesses since they are brought on by interactions between various genes and environmental factors that are still unknown. All three conditions are incurable, and a sizable portion of patients eventually develop end-stage liver disease necessitating a liver transplant. The treatments aim on preventing disease progression and treating the associated symptoms for each disease.