Autoimmune Hepatitis (AIH) is a chronic liver disease that occurs due to continuous hepatocellular inflammation and necrosis.  

Cirrhosis causes from progressive fibrosis with immune serum markers such as autoantibodies and higher IgG levels. 

Autoimmune hepatitis is linked to other autoimmune diseases, but it is not caused due to viruses, alcohol intake or chemicals. 

Types of AIH are: 

Type 1 

Type 2 

Type 1 AIH occurs at any age, and it is diagnosed in young adults and >50 years old. Type 2 AIH is less common, and it is diagnosed in children and teenagers. 

The immune system mistakenly targets liver cells to cause chronic inflammation.  

In 1950, chronic hepatitis in young women is described by Waldenstrom with cirrhosis, and liver plasma cell infiltration. 

It has prevalence in US as 31.2 cases per 100000 individuals. While prevalence is 43 cases per 100000 persons in a native Alaskan population. 

Autoimmune hepatitis is more prevalent in women. In men it affected more than women in older age groups. 

The new onset of autoimmune hepatitis is most reported in the 10 to 30 and 40 to 60 years old. 

Autoimmune hepatitis may occur in people of any age. The diagnosis should not be avoided in individuals older than 70 years old.  

Genetic factors and environmental triggers to cause autoimmune response against liver antigens. 

C4A gene deletions are associated with the development of autoimmune hepatitis. HLA-DR3-positive patients have aggressive liver disease that is treatment-resistant and may require transplantation. 

Autoimmune hepatitis related to complement allele C4AQO causes C4 deficiency. Viral and drug-induced autoimmunity targets drug-metabolizing enzymes. 

Autoimmune hepatitis results from immune tolerance breakdown. Regulatory T cells maintain immune balance. 

Unknown etiology of autoimmune hepatitis triggers autoimmune response and disease. 

It shows good prognosis for autoimmune hepatitis with mild liver inflammation and fibrosis. Type 2 autoimmune hepatitis patients have worse prognosis than type 1. 

Severe initial presentation or inflammation on liver biopsy predicts worse long-term outcomes compared to mild cases. 

Around 50% of patients with jaundice during presentation. Cirrhosis found in 30% to 38% of adults and children with type 2 autoimmune hepatitis. 

Autoimmune hepatitis may present in the form of acute and chronic hepatitis. 

• Skin Examination 
• Abdominal Examination 
• Musculoskeletal Examination 
• Neurological Examination 

Asymptomatic symptoms are: 

Fatigue, mild right upper quadrant discomfort, and joint pain 

Acute symptoms are: 

Jaundice, dark urine, pale stools, anorexia, nausea, vomiting, and right upper quadrant pain 

Asymptomatic patients with autoimmune hepatitis should receive the same treatment as symptomatic patients due to similar outcomes. 

Patients may achieve drug-free remission within a few years, but many require lifelong monitoring and treatment. 

Prednisone doses for adults and children with severe autoimmune hepatitis are appropriate. 

Patients in remission stop immunosuppression after second liver biopsy. 

Budesonide therapy for Crohn’s disease may cause fewer side effects like weight gain and bone density reduction compared to prednisolone. 

Immunosuppressants and ursodiol treatment advised for autoimmune hepatitis-PBC overlap. 

Patients may achieve 4 treatment end points as follows: 

• Remission 
• Treatment failure 
• Incomplete response 
• Drug toxicity 

Gastroenterology

Patient should start healthy diet including fruits and vegetables, grains. 

Enough water and fluids intake on regular basis must be followed by patient during period of recovery. 

Proper awareness about autoimmune hepatitis should be provided and its related causes with management strategies. 

Appointments with a gastroenterologist and preventing recurrence of disorder is an ongoing life-long effort. 

Gastroenterology

Prednisone: 

It stabilizes lysosomal membranes to suppress lymphocytes and antibody production. 

Budesonide: 

It is an anti-inflammatory corticosteroid with potent glucocorticoid activity. 

Gastroenterology

Azathioprine: 

It antagonizes purine metabolism to block DNA replication in T and B lymphocytes. 

Mycophenolate: 

It inhibits the production of inosine phosphate dehydrogenase and guanosine nucleotides. 

Gastroenterology

Paracentesis suggested to relieve symptoms of ascites for signs of infection. 

Liver transplantation involves the surgical removal of the diseased liver and replacement with a healthy donor liver. 

Gastroenterology

In the initial assessment phase, early detection initiates treatment promptly and prevents progression to severe disease. 

Pharmacologic therapy is effective in the treatment phase as it includes use of corticosteroids and immunosuppressants agents. 

In supportive care and management phase, patients should receive required attention such as lifestyle modification and intervention therapies. 

The regular follow-up visits with the gastroenterologist are scheduled to check the improvement of patients along with treatment response.