We speak of 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 organism as happens in healthy subjects, attack and damage the liver.
The precise triggers remain unknown.
Figure: schematic of the attack of auto-antibodies against liver cells. From the site: aboutkidshealth.ca
Some researchers argue 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.
Symptoms of autoimmune hepatitis are numerous and include jaundice, spider angiomas, dark urine, fatigue and amenorrhea (in women).
For a correct diagnosis, blood tests and a liver biopsy are needed.
Permanent recovery is difficult, so much so that corticosteroid and immunosuppressive treatments often last for a lifetime.
What is autoimmune hepatitis?
Autoimmune hepatitis is an inflammation of the liver that arises as a result of an abnormality in the immune system.
In fact, people with autoimmune hepatitis have a poorly functioning immune system that, instead of simply carrying out its normal defensive functions, attacks the liver, damaging it.
Autoimmune hepatitis has all the connotations of a chronic disorder, as the anomaly of the immune system that characterizes it, once it appears, is persistent and capable of lasting for the rest of life.
IMMUNE SYSTEM AND AUTOIMMUNE PATHOLOGIES
The immune system is the defensive barrier of an organism against threats coming from the external environment, such as viruses, bacteria, parasites, etc., but also from the inside, such as for example crazed (tumor) or malfunctioning cells.
The immune system is made up of an "army" of cells and glycoproteins that are very effective and very aggressive towards those who represent a potential danger.
In some individuals, for very often unknown or unclear reasons, the immune system can undergo an alteration that causes it to attack some perfectly healthy cells of the organism, attacking them. All this can cause damage, sometimes even very serious, to various organs and tissues of the body. This abnormal behavior of the immune system distinguishes so-called autoimmune diseases.
TYPES OF AUTOIMMUNE HEPATITIS
Doctors have identified two main types of autoimmune hepatitis:
- Type 1 autoimmune hepatitis, or classic autoimmune hepatitis. It is the most common type; it can arise at any age and in more than 50% of cases it is accompanied by other autoimmune diseases, such as thyroiditis, rheumatoid arthritis and colitis ulcerative.
- Type 2 autoimmune hepatitis 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 together with other autoimmune diseases.
EPIDEMIOLOGY
Autoimmune hepatitis is an uncommon disease: according to a reliable Anglo-Saxon source, in fact, it affects one person in 10,000. It is also more common among women, both as regards type 1 and type 2 autoimmune hepatitis. No differences were found between the various ethnic groups, so it has more or less the same incidence in all. the world.
Causes of autoimmune hepatitis
We have explained how autoimmune hepatitis is due to an anomaly of the immune system, which attacks the liver as if it were a threat to the organism. It remains now to understand what are the causes of this anomaly.
Currently, the precise reasons that "upset" the immune system remain unclear; some researchers argue 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
They are most at risk of autoimmune hepatitis:
- Women
- Those who have contracted certain bacterial or viral infections.
- Those who have used certain medications, 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-familial predisposition is necessary to get sick.
- Those suffering from other autoimmune diseases.
Symptoms, signs and complications
The attack of the immune system, which damages the liver, leads to chronic inflammation and the 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 and sudden onset symptoms, while others suffer from mild disorders with a very gradual onset.
Figure: jaundice
Going into detail, the signs and pathological expressions that distinguish autoimmune hepatitis are:
- Sense of fatigue
- Diffuse abdominal pain
- Joint pain
- Itching
- 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, which can affect blood vessels, lymphatics, and bile vessels. Spider angioma is a typical sign of liver inflammation.
- Nausea and vomit
- Loss of appetite
- Skin rashes of various types. The term rash is synonymous with a 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 pathologies of autoimmune etiology, some even very serious. According to some researchers, this association is consequential (ie there is some connection), but in this regard there is still no concrete evidence.
Related autoimmune diseases are:
- Pernicious anemia. The term anemia indicates the lack of red blood cells. Pernicious anemia occurs when a fundamental factor for the formation of red blood cells is attacked (and destroyed), for no specific reason, by certain cells of the immune system.
- Hemolytic anemia. In patients with hemolytic anemia, the immune system destroys red blood cells and does so at a faster rate than their production.
- Ulcerative colitis. Belonging to the so-called inflammatory bowel 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 rheumatoid arthritis patients, 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. The aggression takes place in the intestine and involves the deterioration of the intestinal walls.
COMPLICATIONS
Left untreated, autoimmune hepatitis can degenerate into cirrhosis.
Cirrhosis is a very serious liver disease characterized by the death and subsequent replacement of healthy liver cells with scar tissue.
- Hepatic hypertension
- Esophageal varices
- Ascites
- Hepatic insufficiency
- Liver cancer
This can have several consequences: a change in blood flow directed to the liver (which then gives rise to so-called portal hypertension and so-called esophageal varices), an abnormal collection of fluid in the peritoneal cavity (ascites), a reduction in liver function (liver failure) and, finally, a liver tumor.
WHEN TO SEE THE DOCTOR?
Since some symptoms of autoimmune hepatitis are the same as those of other less serious and less alarming diseases, sufferers 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, an objective 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 collect a small sample of cells from the patient. liver diseases (liver biopsy).
BLOOD TESTS
Antibodies, or immunoglobulins, constitute a department of the immune army. These special proteins, under normal conditions, fight only the threats that come from the external environment, while in situations such as autoimmune hepatitis they involuntarily become the main culprits of inflammation of the liver. Once called to act, antibodies take on different characteristics depending on the enemy or, in the case of an autoimmune disease, on the organ they attack.
The blood of a person with autoimmune hepatitis contains special 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 trace the precise cause of the liver inflammation and to rule out 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 way to diagnose hepatitis and to establish its causes and severity. The procedure is slightly invasive, as a fairly large needle is introduced where the liver is located.
Therapy
The only way to counteract the effects of autoimmune hepatitis (be it type 1 or type 2) in any way is to slow down, if not stop, the adverse reaction set in motion by the immune system. To achieve this therapeutic goal, different categories of drugs are being helped, such as corticosteroids and immunosuppressants.
If, unfortunately, the drug treatment fails and liver inflammation leads to severe cirrhosis, liver transplantation becomes fundamental for the patient's survival. Unfortunately, even with the appropriate treatments, the chances of a complete recovery from hepatitis autoimmune are very poor.
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 beginning of treatment, it is administered in high doses; then, over the weeks, it is gradually reduced until the minimum effective dose is reached, which is maintained for at least 18-24 months. In several cases, given the chronicity of the disease , the recruitment can also last a lifetime.
Unfortunately, prolonged intake of prednisone (or any other corticosteroid) can cause serious side effects, such as diabetes, osteoporosis, hypertension, cataracts, weight gain, etc. - Azathioprine. Azathioprine is an immunosuppressant, that is, 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, in order to reduce the doses of the latter.
Those who have a weaker immune system are 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 banal seasonal flu).
Azathioprine treatment can also last a lifetime.
If prednisone and / or azathioprine are ineffective, more potent immunosuppressants such as mycophenolate, cyclosporine and tacrolimus can be used.
Attention: a clear improvement in symptoms does not necessarily mean being cured of autoimmune hepatitis. Therefore, in the presence of even a significant reduction in symptoms, it is not recommended to stop pharmacological treatments without a precise indication from the doctor.
LIVER TRANSPLANTATION
In the presence of autoimmune hepatitis, a liver transplant is indicated when drug treatments have not given the desired results and when the patient suffers from liver failure (severe liver cirrhosis).
Thanks to the extraordinary self-healing abilities of the liver, the person from whom the liver is taken can also be a living individual (N.B: in these cases, obviously, the entire 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 you suffer from involves.
- Eat healthy and exercise (obviously suited to your health).
- Do not drink alcohol for any reason.
- Do not interrupt treatments, unless on medical advice.
- Request the support of friends and family.
- Contact some support group for hepatitis patients.
Prognosis
Autoimmune hepatitis is a chronic disorder that strongly affects the quality of life of patients and from which it is rare to recover permanently.
Typically, sufferers are forced to take medications (prednisone and azathioprine) for long periods of time, if not for a lifetime.
Furthermore, when drug treatments fail, the situation becomes even more complicated, autoimmune hepatitis degenerates into cirrhosis and a liver transplant is required.
