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Hepatologist and Liver Transplant | Dr Manas Vaishnav

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Autoimmune Hepatitis

Autoimmune hepatitis (AIH) is a chronic liver disease where the body’s immune system attacks its own liver cells. This causes inflammation and, if untreated, can lead to progressive liver damage. The exact cause isn’t fully understood – it’s thought to be a combination of genetic predisposition and some environmental trigger that misdirects the immune system. Autoimmune hepatitis is relatively rare. It can occur at any age and in any ethnicity, but it is most common in young to middle-aged women. Unlike viral hepatitis, AIH is not contagious. It often presents insidiously, and because symptoms can be mild, diagnosis is sometimes delayed until significant liver injury has occurred.

(For simplicity, we will use MASLD to refer to this condition, in line with the newer terminology, but many doctors and patients may still know it as NAFLD.)

Symptoms

Symptoms of autoimmune hepatitis range from none at all to signs of acute hepatitis. Many patients have a gradual onset of symptoms. Common manifestations include:

  • Fatigue and lethargy: Feeling very tired is one of the most frequent (and often overlooked) symptoms. Patients may attribute it to stress or aging.
  • General discomfort or mild upper abdominal pain: Some have a persistent ache or heaviness over the liver area.
  • Jaundice: Yellowing of the skin and eyes occurs in about half of cases at presentation. Patients might notice dark urine or pale stool associated with this. Sometimes jaundice in AIH can be fluctuating – appearing, then improving, then returning.
  • Joint pains and muscle aches: AIH can have systemic symptoms; some patients report aching joints or even arthritis-like symptoms.
  • Skin manifestations: Occasionally rashes or acne may flare with AIH activity. Some develop small blood vessel lesions on the skin (“spider angiomas”) or other signs of liver disease if advanced (like redness of the palms).
  • Other nonspecific signs: Loss of appetite, nausea, or mild abdominal swelling can occur. Some women with AIH have a history of irregular menstrual cycles or amenorrhea (loss of periods) when the disease is active.

It’s worth noting that not all patients have symptoms. Some are discovered because of abnormal liver blood tests on routine exams. AIH can also sometimes present like an acute hepatitis with sudden onset of jaundice and illness (including potentially acute liver failure), though this is less common.

If AIH has progressed to cirrhosis by the time of diagnosis, there may be signs of advanced liver disease: fluid in the abdomen (ascites), mental confusion (encephalopathy), easy bruising, etc. But catching it earlier is preferable.

Causes

The cause of autoimmune hepatitis is essentially an overactive immune response targeting the liver. The immune system, which normally defends against viruses and bacteria, mistakenly recognizes liver cells as foreign and attacks them. Why this happens isn’t fully clear, but likely due to a mix of factors:

  • Genetic predisposition: Certain genes that regulate immune function are more common in people with AIH. For instance, particular HLA genotypes (part of the immune system) are associated with AIH. This suggests heredity plays a role, as AIH can run in families to some degree.
  • Environmental triggers: It’s suspected that in someone susceptible, a trigger like a viral infection, certain medications, or other environmental exposures might “trigger” the autoimmune process. Examples proposed include viruses like measles, hepatitis viruses, Epstein-Barr, or drugs such as minocycline or nitrofurantoin (which in rare cases cause an AIH-like syndrome). However, no definitive trigger is identified in most cases.
  • Other autoimmune conditions: Many people with AIH also have other autoimmune diseases, or there’s a family history of them. This connection suggests a general propensity for autoimmunity. Common associations include thyroid autoimmune diseases (like Hashimoto’s thyroiditis), rheumatoid arthritis, celiac disease, ulcerative colitis (especially in type 2 AIH), or type 1 diabetes.
  • Type 1 AIH: The most common form. Can occur at any age (often young adult women). Often associated with other autoimmune diseases. Blood tests typically show ANA (antinuclear antibodies) or SMA (smooth muscle antibodies).
  • Type 2 AIH: Less common, tends to affect children/teens or young adults. Often has anti-LKM1 (liver kidney microsomal) antibodies. Can be more aggressive and also associated with other immune issues.

Regardless of type, the fundamental cause is the body’s immune T-cells targeting liver cells. This results in inflammation (hepatitis). If persistent, it causes scarring (fibrosis) and can eventually lead to cirrhosis.

Diagnosis

Diagnosing autoimmune hepatitis involves a combination of clinical suspicion, blood tests, and sometimes liver biopsy:

  • Liver function tests: Typically, AIH causes elevated liver enzymes, especially ALT and AST (often very high, in the hundreds). It usually shows a hepatocellular pattern (ALT/AST much higher than alkaline phosphatase). Bilirubin may be elevated if there is jaundice.
  • Autoantibody tests: These are crucial. Common autoantibodies in AIH include:
    • ANA (Antinuclear Antibody): Often positive in type 1 AIH (not specific, since also positive in lupus and other diseases).
    • SMA (Smooth Muscle Antibody): Common in type 1 AIH.
    • LKM-1 (Liver Kidney Microsomal Antibody): Seen in type 2 AIH, more in pediatric cases.
    • Anti-SLA/LP (Soluble Liver Antigen): Another antibody that can be present.

    About 80–90% of AIH patients have at least one of these antibodies positive.

  • Immunoglobulin levels: Total IgG in blood is often elevated in AIH. High IgG supports the diagnosis since the immune system is activated.
  • Excluding other causes: Doctors will test for viral hepatitis (A, B, C, etc.) and check for drug/alcohol use. If those are negative and autoantibodies are positive, AIH is suspected.
  • Liver biopsy: Usually done to confirm diagnosis and assess severity. Typical findings: interface hepatitis (immune cells attacking liver cells at junction of lobules) and plasma cell prominence. Biopsy also shows fibrosis/cirrhosis level, guiding treatment urgency.

Doctors also use diagnostic scoring systems combining these factors (the International Autoimmune Hepatitis Group has one) to establish the diagnosis.

Often, by the time it’s diagnosed, patients might already have significant inflammation or even early cirrhosis, because symptoms can be subtle for a while. The key is that AIH is quite treatable if caught.

Treatment

The goal in AIH is to suppress the immune attack on the liver, thereby reducing inflammation and preventing progression to cirrhosis. The standard treatment regimen has been in use for decades and is quite effective in most cases:

  • Corticosteroids (Prednisone): First-line therapy. Prednisone (or prednisolone) is started at a relatively high dose (30–40 mg daily) to quickly reduce inflammation. As liver enzymes normalize and symptoms improve, the dose is tapered to the lowest effective level. Steroids often induce remission within weeks, but long-term high doses cause side effects, so tapering is important.
  • Azathioprine (Imuran): An immunosuppressant often added as a steroid-sparing agent. Strategy: start prednisone, then add azathioprine once the patient responds, allowing prednisone tapering. Azathioprine alone or with low-dose prednisone can maintain remission with fewer side effects. If intolerant (e.g., low white cell counts or side effects), alternatives like mycophenolate mofetil may be used.
  • Duration of therapy: Treatment is usually long-term. After remission (normal labs and improved biopsy), therapy continues for 2–3 years before considering withdrawal. Many patients relapse if meds are stopped. If cirrhosis is present, treatment is typically lifelong. Patients with ≥2 relapses after stopping usually need indefinite maintenance therapy.
  • Other medications: In difficult or non-responsive cases:
    • Mycophenolate, tacrolimus, or cyclosporine – alternative immunosuppressants.
    • Budesonide: A steroid with high first-pass metabolism, used instead of prednisone in milder, non-cirrhotic cases to reduce systemic side effects.
    • Biologics: Under research, but not yet routine.

With proper treatment, about 80% of patients can achieve remission. It’s critical to adhere to meds and follow regularly with a doctor. Liver enzymes and IgG levels are monitored to gauge response, and sometimes a repeat biopsy is done after a couple years to confirm that inflammation is gone before attempting to stop treatment.

If autoimmune hepatitis is not treated, it can lead to cirrhosis and possibly liver failure. In end-stage cases, or those rare fulminant cases, liver transplantation is an option. Transplant yields excellent results for AIH patients, though the underlying tendency for autoimmunity can occasionally cause the hepatitis to recur in the new liver (usually milder).

FAQs

We usually say it’s treatable rather than “curable.” With medication, AIH can be driven into remission (meaning the liver inflammation goes away and liver tests normalize) in the majority of patients. In that sense, the disease can be controlled almost completely. However, the underlying tendency of your immune system to attack the liver often persists. Many patients need long-term or lifelong medication to keep the disease quiescent. Some can taper off meds after a few years of remission, but about 70-80% of those will experience a relapse of hepatitis at some point and have to restart treatment. Think of it like hypertension or diabetes – a chronic condition that you manage: the meds keep it in check, but if you stop them without careful consideration, the problem can come back. There are cases (especially if caught very early) where treatment for a few years leads to a long-lasting remission off drugs, but it’s not guaranteed. The key is, with ongoing treatment, people with AIH can often lead normal lives and the liver can heal.

The standard meds, prednisone and azathioprine, do have potential side effects. Prednisone, especially at high doses or long-term, can cause weight gain, a round face, acne, mood swings, insomnia, osteoporosis (bone thinning), diabetes, high blood pressure, cataracts, among others. That’s why doctors aim to taper it to the lowest dose possible or off if they can. Azathioprine can cause low blood counts, nausea, and rarely liver toxicity or pancreatitis. It also carries a small long-term risk of malignancy (like lymphoma) after many years, but at the doses used for AIH this risk is quite low. Despite these concerns, it’s important to remember that doing nothing (leaving AIH untreated) is much riskier to your health than the medications. Doctors will monitor you – for example, regular blood tests to check blood cell counts and liver enzymes while on azathioprine, and guidelines to mitigate steroid side effects (like calcium/Vitamin D for bone health, etc.). Many people tolerate the meds quite well, especially once on low-dose maintenance. If someone absolutely cannot handle a medication, alternative drugs can be tried as mentioned. Open communication with your doctor about side effects is key, as adjustments can often be made to find a tolerable regimen.

Autoimmune hepatitis is not contagious at all – it cannot be passed from person to person. It’s your own immune system acting up, not an infection. So you don’t need to isolate or worry about infecting anyone. However, there is a bit of a genetic tendency. Family members (especially first-degree relatives) might have a slightly higher likelihood of having autoimmune diseases in general. They do not need screening for AIH specifically unless they have abnormal liver tests or symptoms. It’s relatively rare to find more than one person in a family with AIH, but other autoimmune illnesses (thyroid, rheumatoid, etc.) might crop up in family members due to shared genetic background. In short: no special testing for relatives is recommended just because you have AIH, unless they have health issues that warrant it. Focus on your own health and treatment, and reassure family that they can’t “catch” this from you.

It is strongly advised to avoid alcohol or keep it extremely minimal. Your liver is already dealing with inflammation; alcohol is another stressor that can further damage liver cells. While a tiny occasional drink might not kill you, the safest approach for someone with any liver disease (especially active hepatitis or cirrhosis) is to abstain completely. Also, if you’re on medications like azathioprine, alcohol can compound liver strain. Consider that one of the key goals is to give your liver the best chance to heal – alcohol would be counterproductive to that. So, it’s best to cut out alcohol entirely or discuss with your hepatologist if any amount is permissible in your specific case (many will say zero, some might allow rare special-occasion small drinks if your disease is in full remission and liver function is normal, but even then it’s cautious). When in doubt, avoiding alcohol is the safest course.

The prognosis has improved greatly, thanks to therapy. If diagnosed early and treated properly, many patients achieve remission and have a normal life expectancy. The liver can heal much of the damage if inflammation is stopped. Studies show that the 10-year survival of patients in remission can approach that of the general population. However, if the disease is aggressive and not treated, or if it’s only discovered at a very late stage (when cirrhosis is already advanced), it can be life-threatening – untreated AIH has a high 5-year mortality historically. The worst outcomes tend to be in those who either don’t respond to medications or who already had cirrhosis and complications at diagnosis. Even in cirrhosis, treatment improves outlook significantly. Some patients with very advanced disease may require a liver transplant; outcomes after transplant for AIH are excellent (over 80–90% 5-year survival post-transplant). AIH can recur in a transplanted liver, but usually that’s manageable and less aggressive. Overall, with compliance to treatment and regular follow-ups, most people with AIH do very well.