Related Subjects:
|Chronic liver disease
|Cirrhosis
|Alkaline phosphatase (ALP)
|Liver Function Tests
|Ascites Assessment and Management
|Budd-Chiari syndrome
|Autoimmune Hepatitis
|Primary Biliary Cirrhosis
|Primary Sclerosing Cholangitis
|Wilson disease
|Hereditary Haemochromatosis
|Alpha-1 Antitrypsin (AAT) deficiency
|Non alcoholic steatohepatitis (NASH)
|Spontaneous Bacterial Peritonitis
|Alcoholism and Alcoholic Liver Disease
|Liver Transplantation
Autoimmune Hepatitis (AIH) is an autoimmune attack on healthy liver cells, leading to inflammation and, over time, can result in liver damage, fibrosis, and cirrhosis.
About
- Liver inflammation suspected with negative viral serology and lack of a toxin and the presence of autoantibodies
Clinical
- Most commonly found in young and middle-aged women
- Fatigue and mild to moderate jaundice and chronic liver disease.
- Hepatosplenomegaly
Types
- Types 1 "classical and commoner" Anti SMA/ANA
- This is the most prevalent form, occurring in both adults and children.
- +ve antinuclear antibody and anti-dsDNA and even +ve ANCA
- +ve anti smooth muscle (Anti-Actin Antibodies AAA)
- Strong association with other autoimmune diseases.
- Type 2 Ant LKM1 less common but often more aggressive
- Less Common: More commonly diagnosed in children and young adults.
- +ve Anti Liver Kidney Microsomal (Anti-LKM1)
- Main target is Cytochrome P4502D6 on liver plasma membranes
- Girls and young women present with acute hepatitis
Clinical
- Malaise, fatigue, jaundice, vomiting, anorexia
- Hepatitis : RUQ pain, jaundice
- Serum sickness: Arthralgia, polyarthritis, rash, dry eyes
- Signs of progressive liver disease
- Dark Urine and Pale Stools: Symptoms of liver dysfunction.
Associations
- Hashimoto's thyroiditis
- Renal tubular acidosis
- Rheumatoid arthritis
- Type 1 Diabetes, Amenorrhoea, Acne
- Sjogren's syndrome, Vitiligo
Investigations
- Anaemia, High WCC, low plt, Prolonged Prothrombin time, Raised ESR
- Elevated AST, ALT, ALP and Bilirubin
- Antibodies: ANA, SMA, LKM-1 helps in diagnosing and differentiating the type of AIH.
- IgG polyclonal rise
- Liver Biopsy - interface hepatitis, There is periportal piecemeal and bridging necrosis on liver biopsy and cirrhosis
- Imaging: Ultrasound, CT scan, or MRI may be used to assess liver structure and rule out other causes of liver disease.
Complications
- End stage liver disease
- Cirrhosis and its complications ascites, varices etc
Management
- Prednisolone 30 mg for 2 weeks then slowly reducing the dose. Effective in most patients and improves survival.
- Azathioprine improves prognosis and is used as a steroid-sparing agent (Test for Thiopurine methyltransferase activity first)
- Ursodeoxycholic acid has been used
- Liver transplantation in end stage liver disease
- Vaccinate against Hepatitis A and B
- USS/AFP screening for hepatocellular carcinoma