Autoimmune hepatitis: what it is, causes, treatment

Sixteen to eighteen people develop autoimmune hepatitis per 100,000 inhabitants, according to Filfoie, the rare liver disease health network. Women are the main victims: there is indeed one man for every seven women among the patients listed.

Although children can be affected, sometimes even before puberty, the majority of patients are diagnosed between the ages of 40 and 60.

In the absence of treatment, the liver deteriorates gradually until it generates cirrhosis, even acute liver failure requiring a liver transplant in the most severe cases. Autoimmune hepatitis is thus the cause of 3 to 4% of liver transplants in Europe. This condition should therefore not be taken lightly.

Causes still unclear

It is not known why this hepatitis occurs suddenly. But as in many autoimmune diseases, it appears in patients with a background that predisposes them to an immune disorder. Their natural defense system chooses the wrong enemy.

It considers this organ as an intruder and begins to manufacture antibodies directed against it. Result: the liver becomes inflamed and changes its architecture, which prevents it from functioning properly. “Other autoimmune pathologies are often present in the family, such as type 1 diabetes, Hashimoto’s thyroiditis or rheumatoid arthritis”, underlines Professor Didier Samuel, head of the Hepatology and Resuscitation Unit. Hepatic at the Paul-Brousse hospital (Villejuif).

Triggering factors are certainly also involved. Prior infection with a virus is strongly suspected, since many autoimmune hepatitis are preceded by measles, chicken pox or even mononucleosis.

Certain drugs are also in the hot seat, in particular Oxyphenisatin, used in the treatment of constipation, hypertension, certain antibiotics and some anti-inflammatories known to attack the liver.

“Relapses can also occur after childbirth, insofar as pregnancy induces a particular immune state so that the mother’s body tolerates the fetus”, observes Professor Didier Samuel.

Autoimmune hepatitis: the warning signs

Everything often starts with a routine or prescribed blood test in case of unexplained severe fatigueof bulky liver or of pains.

When the results come in, one of the first warning signs is high levels of transaminases (ASAT and ALAT), biological markers that increase during the destruction of liver cells. These can climb more than 5 times above normal!

When the disease has already progressed, yellowing of the skin and the white of the eye (jaundice) may additionally be observed. A heavy consumption of alcohol, drugs or viral hepatitis are likely to generate the same characteristics. But in the event of suspicion of autoimmune hepatitis – by eliminating other possible causes -, a search for specific antibodies is carried out in a specialized medical analysis laboratory in order to validate the hypothesis.

Biopsy, an essential examination to diagnose autoimmune hepatitis

“As autoimmune hepatitis generally evolves quietly, the liver of many newly diagnosed patients already shows lesions”, observes Professor Samuel. To assess their magnitude and determine the most appropriate management, a biopsy is therefore essential.

The initial workup often also includes an MRI of the bile ducts, as these can also be affected.

“It all started for me in 2019. My GP was surprised by my high level of transaminases, says Olga, a 39-year-old teacher. It was only 3 times higher than normal at the time, but given my family history of autoimmune diseases, he asked me for an antibody test. As it came back positive and my transaminases continued to rise, I had to undergo many other tests in 2020. My biopsy was deprogrammed twice in a row due to the Covid epidemic. When I was finally able to do it, my liver was already badly damaged.”

What treatments for autoimmune hepatitis?

“For optimal care, it is better to be followed in a hospital center specializing in inflammatory diseases of the liver and bile ducts, which are spread throughout the territory.

The most common initial treatment involves taking two medications: first a corticosteroid to soothe inflammationthen an immunosuppressantl’azathioprine, to avoid the destruction of the liver.

In 80% of cases, this dual therapy is very effective. The doses of corticosteroids can be reduced in stages as soon as the level of transaminases normalizes, but the immunosuppressant must be maintained for several years, sometimes even for life because recurrences are frequent.

The sky fell on my head when I was diagnosed with autoimmune hepatitis. My biological balance is stabilized today but with the stop of cortisone, I am again very tired, testifies Elisa, photographer of 45 years. I can’t concentrate on anything anymore. Fortunately, my companion takes care of our two children, because I have no more energy. I also have joint pain and muscle burns that wake me up at night. I know these symptoms will eventually go away, but it’s hard to live with on a daily basis. It seems that hypnosis and the practice of a sport improve things. I will try soon.”

For very severe forms, a liver transplant is required.

A degraded liver can lead to a fatal coma. “In the event of an acute fulminant attack, resistant to high-dose treatment, liver transplantation becomes the only recourse”, explains Professor Didier Samuel. It is the same in case of hepatic insufficiency due to an important cirrhosis.

Autoimmune hepatitis represents 5% of transplants for cirrhosis. After transplantation, the survival rate is 80% at two years. Recurrences are frequent (50% of patients), but rarely lead to the loss of the graft, according to a 2019 study from the CHU de Lyon.

If you or someone close to you has autoimmune hepatitis, you can get help from the Albi patient association. If a liver transplant is being considered, turn to the Federation of liver patients and transplant recipients (Transhepate).