autoimmune diseases

Autoimmune hepatitis

We talk about autoimmune hepatitis when the liver is affected by an inflammatory process, due to the attack of abnormal immune cells. These cells, instead of defending the body as in healthy subjects, attack and damage the liver.

The precise triggering causes remain unknown.

Figure: schematization of the attack of auto-antibodies against liver cells. From the site: aboutkidshealth.ca

Some researchers claim that some factors play a fundamental role; among these, the most studied are genetic predisposition, direct contact with certain infectious agents and the intake of particular drugs.

The symptoms of autoimmune hepatitis are numerous and include jaundice, appearance of spider angiomas, dark urine, sense of fatigue and amenorrhea (in women).

For a correct diagnosis, blood tests and a liver biopsy are required.

Permanently healing is difficult, so much so that often corticosteroid and immunosuppressive treatments last for life.

What is autoimmune hepatitis?

Autoimmune hepatitis is an inflammation of the liver that occurs following an abnormality of the immune system .

People with autoimmune hepatitis, in fact, have an immune system that works badly and that, instead of simply performing its normal defensive functions, attacks the liver, damaging it.

Autoimmune hepatitis has all the characteristics of chronic disorder, as the abnormality of the immune system that characterizes it, once it appears, is persistent and able to last for the rest of life.

IMMUNE SYSTEM AND AUTOIMMUNE PATHOLOGIES

The immune system is an organism's defensive barrier against threats from the external environment, such as viruses, bacteria, parasites etc., but also from within, such as, for example, cells that have gone mad (cancer) or malfunctioning.

The immune system is made up of an "army" of very effective and very aggressive cells and glycoproteins against those who represent a potential danger.

In some individuals, for very often unknown or unclear reasons, the immune system may undergo an alteration which causes it to attack some perfectly healthy cells of the organism, attacking them. All this can lead to damage, sometimes very serious, to different organs and tissues of the body. This abnormal behavior of the immune system distinguishes the so-called autoimmune diseases .

TYPES OF AUTOIMMUNE HEPATITIS

Doctors have identified two main types of autoimmune hepatitis:

  • Autoimmune hepatitis type 1, or classical autoimmune hepatitis . It is the most common type; it can occur at any age and in more than 50% of cases it is accompanied by other autoimmune diseases, such as thyroiditis, rheumatoid arthritis and ulcerative colitis.
  • Type 2 autoimmune hepatitis . It is the most common type among young people (especially women) and is generally more severe than the previous one. Similar to type 1, it also occurs along with other autoimmune diseases.

Epidemiology

Autoimmune hepatitis is an uncommon disease: according to a reliable Anglo-Saxon source, in fact, it affects one person every 10, 000. It is also more common among women, both in terms of autoimmune hepatitis type 1 and type 2. There is no difference between the various ethnic groups, so it has more or less the same incidence in all the world.

Causes of autoimmune hepatitis

We explained how autoimmune hepatitis is due to an abnormality of the immune system, which attacks the liver as a threat to the body. It remains now to understand what the causes of this anomaly are.

Currently, the precise reasons that "upset" the immune system remain unclear; some researchers claim that autoimmune hepatitis is the result of a combination of several factors, including a certain genetic-familial predisposition, contact with certain infectious agents and the intake of particular drugs.

RISK FACTORS

I am more at risk of autoimmune hepatitis:

  • Women
  • Those who have contracted certain bacterial or viral infections.
  • Those who have used certain drugs, such as minocycline (an antibiotic) and atorvastatin (a medicine used to lower cholesterol).
  • Those who have a parent or sibling with the same disease. This has led researchers to think that a particular genetic-family predisposition is necessary to get sick.
  • Those who suffer from other autoimmune diseases.

Symptoms, signs and complications

The aggression of the immune system, to the detriment of the liver, involves chronic inflammation and deterioration of liver cells. The manifestations of this damage can be more or less serious and more or less sudden: some patients, in fact, suffer from severe symptoms and sudden onset, while others suffer from mild onset which is very gradual.

Figure: jaundice

Going into detail, the signs and pathological expressions that characterize autoimmune hepatitis are:

  • Sense of fatigue
  • Widespread abdominal pain
  • Joint pain
  • itch
  • Jaundice . In the presence of jaundice, the skin and sclera of the eyes take on a yellowish color. This is due to an increase in bilirubin levels in the blood.
  • Enlarged liver
  • Spider angiomas . An angioma is a mostly benign tumor that can affect blood vessels, lymphatic vessels and bile vessels. Spider angioma is a typical sign of liver inflammation.
  • Nausea and vomit
  • Loss of appetite
  • Various types of skin rash. The term rash is synonymous with rash or rash.
  • Dark urine
  • Amenorrhea in women. Amenorrhea is the lack of menstruation.

ASSOCIATED AUTOIMMUNE DISEASES

In many patients, autoimmune hepatitis is associated with other autoimmune diseases, some even very serious. According to some researchers, this association is consequential (that is, there is some connection), but in this regard there is still no concrete evidence.

The related autoimmune diseases are:

  • Pernicious anemia . The term anemia indicates the lack of red blood cells. Pernicious anemia occurs when a key factor in the formation of red blood cells is attacked (and destroyed) by some cells of the immune system for no specific reason.
  • Hemolytic anemia . In patients with haemolytic anemia, the immune system destroys red blood cells and does so at a speed higher than their production.
  • Ulcerative colitis . Belonging to so-called intestinal inflammatory diseases, it affects the large intestine and causes diarrhea and abdominal pain.
  • Autoimmune thyroiditis (or Hashimoto's thyroiditis ). The target of the immune system, in this case, is the thyroid gland.
  • Rheumatoid arthritis . The immune system, in patients with rheumatoid arthritis, attacks the joints, causing pain, swelling, stiffness and various motor disabilities.
  • Celiac disease . Celiac disease is caused by an adverse reaction to gluten (a protein found in many cereals), which is inexplicably attacked by the cells of the immune system. Aggression occurs in the intestine and leads to deterioration of the intestinal walls.

COMPLICATIONS

If left untreated, autoimmune hepatitis can degenerate into cirrhosis .

Cirrhosis is a very severe liver disease characterized by death and subsequent replacement of healthy liver cells with scar tissue.

Possible consequence of liver cirrhosis

  • Liver hypertension
  • Esophageal varices
  • Ascites
  • Hepatic failure
  • Hepatic tumor

This can have different consequences: a change in the blood flow to the liver (which then gives rise to the so-called portal hypertension and to the so-called esophageal varices ), an abnormal collection of fluid at the level of the peritoneal cavity ( ascites ), a reduction in liver functions ( liver failure ) and, finally, a liver tumor .

WHEN TO REFER TO THE DOCTOR?

Because some symptoms of autoimmune hepatitis are the same as other less serious and less alarming diseases, it happens that patients do not always realize what they are suffering from.

However, manifestations such as jaundice, dark urine, spider angiomas and amenorrhea are all indicative of a pathological disorder that deserves to be analyzed with appropriate diagnostic tests.

Diagnosis

To diagnose autoimmune hepatitis, the physical examination (ie the analysis of the signs and symptoms complained of by the patient) is not enough. In fact, it is necessary to analyze the composition of the patient's blood and to collect from it a small sample of liver cells (liver biopsy).

BLOOD TESTS

Antibodies, or immunoglobulins, constitute a department of the immune army. These special proteins, under normal conditions, only fight the threats that come from the external environment, while in situations like autoimmune hepatitis they involuntarily become the main culprits of inflammation of the liver. The antibodies, once called to act, take on different characteristics depending on the enemy or, in the case of an autoimmune disease, of the organ that attack.

The blood of a person with autoimmune hepatitis contains particular antibodies, very different from the antibodies present in the blood of a person with viral hepatitis. This allows those who analyze the blood content to go back to the precise cause of liver inflammation and to exclude other causes.

HEPATIC BIOPSY

Liver biopsy consists of the collection and subsequent analysis, in the laboratory, of a small sample of liver cells.

This test is the best method to diagnose hepatitis and to determine its causes and severity. The procedure is slightly invasive, because, where the liver is located, a fairly large needle is introduced.

Therapy

The only way to oppose, in some way, the effects of autoimmune hepatitis (whether type 1 or type 2) is to slow down, or even stop, the adverse reaction implemented by the immune system. To achieve this therapeutic goal, different categories of drugs, such as corticosteroids and immunosuppressants, are helpful.

If, unfortunately, the pharmacological treatment fails and the hepatic inflammation leads to a severe cirrhosis, the liver transplant becomes fundamental for the survival of the patient. Unfortunately, even with the appropriate treatments, the chances of a complete recovery from autoimmune hepatitis are very low.

PHARMACOLOGICAL TREATMENTS

The main drugs administered for the treatment of autoimmune hepatitis are:

  • Prednisone . Prednisone is a powerful anti-inflammatory, belonging to the category of corticosteroid drugs. At the start of treatment, it is given in high doses; then, with the passing of the weeks, it is gradually reduced until the minimum effective dose is reached, which is maintained for at least 18-24 months. In many cases, given the chronic nature of the disease, the intake can last even a lifetime.

    Unfortunately, prolonged intake of prednisone (or any other corticosteroid) can cause serious side effects, such as diabetes, osteoporosis, hypertension, cataracts, increased body weight, etc.

  • Azathioprine . Azathioprine is an immunosuppressant, a drug that lowers the immune system. It is taken to slow down the damage done to the liver by antibodies and other cells of the immune system. Often, it is prescribed in combination with prednisone, so as to reduce the doses of the latter.

    Who has a weaker immune system is more fragile and prone to infections, therefore those who take azathioprine (or any other immunosuppressant) must be careful not to frequent too crowded environments or people suffering from some infectious disease (for example, even a trivial seasonal flu).

    The azathioprine treatment can also last a lifetime.

If prednisone and / or azathioprine are ineffective, more powerful immunosuppressants can be used, such as mycophenolate, cyclosporine and tacrolimus .

Warning: a clear improvement in symptoms does not necessarily mean being healed by autoimmune hepatitis. Therefore, in the presence of an even considerable reduction in symptoms, it is not recommended to interrupt the pharmacological treatments, without a precise indication of the doctor.

LIVER TRANSPLANTATION

In the presence of autoimmune hepatitis, a liver transplant is indicated when pharmacological treatments have not given the desired results and when the patient suffers from liver failure (severe liver cirrhosis).

Liver transplantation is the surgical procedure that replaces a liver that is irremediably damaged with another healthy one, coming from a compatible donor.

Thanks to the extraordinary self-healing abilities of the liver, the person from whom the liver is taken can also be a living individual (NB: in these cases, obviously, the whole organ is not extracted, but only a small portion).

SOME ADVICES

Since autoimmune hepatitis is a chronic condition that is difficult to accept, the doctor advises the patient, for his own good, to:

  • Find out what the disease is suffering from.
  • Eat healthy and do physical activity (obviously consistent with your state of health).
  • Don't drink alcohol for any reason.
  • Do not interrupt the treatments, except on medical advice.
  • Request the support of friends and family.
  • Contact some support group for hepatitis sufferers.

Prognosis

Autoimmune hepatitis is a chronic disorder, which strongly affects the quality of life of patients and from which it is rare to recover permanently.

Generally, patients are forced to take drugs (prednisone and azathioprine) for long periods of time, if not for life.

Moreover, when the pharmacological treatments fail, the situation becomes even more complicated, autoimmune hepatitis degenerates into cirrhosis and a liver transplant is required.