Autoimmune Hepatitis (AIH) is a rare, chronic inflammatory liver disease where the body’s own immune system attacks the liver cells, causing inflammation (hepatitis) and damage. This condition is characterized by elevated liver enzyme levels and increased immunoglobulin G (IgG). If not diagnosed and managed early, this chronic inflammation can lead to progressive cirrhosis (scarring of the liver) and ultimately, liver failure. While the precise cause is unknown, AIH is thought to be triggered by a combination of genetic and environmental factors. Early intervention is critical to prevent irreversible liver damage.

Types of Autoimmune Hepatitis

AIH is classified into two main types based on the presence of specific autoantibodies:

• Type 1 AIH: This is the most common form. It often affects young women and is frequently associated with other co-occurring autoimmune diseases.
• Type 2 AIH: This is the rarer form, typically affecting girls between the ages of 2 and 14.

Clinical Presentation and Symptoms

Symptoms of AIH vary widely in both type and severity among patients. A significant percentage of patients with other autoimmune conditions face an increased risk of developing AIH.

Some individuals may be asymptomatic in the early stages, with symptoms appearing suddenly as the disease progresses. Common signs and symptoms include:

• Systemic: Severe fatigue, joint pain (arthralgia), and loss of menstrual periods (amenorrhea).
• Liver-Specific: Enlarged liver (hepatomegaly), yellow skin and eyes (jaundice).
• Other: Skin rashes, abnormal blood vessels visible on the skin (spider angiomas), abdominal distention, dark-coloured urine, and pale-coloured stools.

Risk Factors for Autoimmune Hepatitis

Certain factors can increase an individual’s susceptibility to developing AIH:

1. Gender: The disease is significantly more common in females than in males.
2. Heredity: A family history suggests a genetic predisposition, indicating an increased risk for close relatives.
3. Infectious History: A history of certain infections, such as those caused by the measles, herpes simplex, or Epstein-Barr virus, may elevate the risk. AIH has also been linked to past infections with Hepatitis A, B, and C viruses.

Diagnosis and Therapeutic Management

The diagnostic process aims to confirm the diagnosis of AIH and assess the extent of liver damage to guide treatment.

Diagnosis

• Blood Tests: Used to measure liver enzyme levels, identify specific autoantibodies, and rule out viral causes of hepatitis.
• Liver Biopsy: Considered the gold standard, this procedure determines the degree and type of liver damage and confirms the diagnosis.

Treatment

The goal of treatment is to suppress the immune response attacking the liver, induce remission, and prevent disease progression.

1. Immunosuppressive Medication:
   • Prednisone: A corticosteroid typically initiated at a high dose to rapidly control the disease, with the dose gradually lowered (tapered) over time.
   • Treatment efficacy is monitored through regular blood tests to track the decline in liver enzyme levels. A positive response may be confirmed with a repeat liver biopsy.
   • Early treatment is vital to reduce the risk of developing cirrhosis and other complications.
2. Liver Transplant:
   • If medical therapy fails to control the disease, and the patient develops end-stage liver failure or advanced, irreversible scarring, a liver transplant becomes necessary.

Autoimmune Hepatitis is often a chronic, lifelong condition that requires sustained patient monitoring, especially following the cessation of medication, to prevent relapse and maintain long-term liver health.