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
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"
- +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
- +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
Associations
- Hashimoto's thyroiditis
- Renal tubular acidosis
- Rheumatoid arthritis
- Type 1 Diabetes,Amenorrhoea, Acne
- Sjogrens syndrome, Vitiligo
Investigations
- Anaemia, High WCC, low plt, Proloned Prothrombin time, Raised ESR
- Elevated AST, ALT, ALP and Bilirubin
- Antibodies as shown above.
- IgG polyclonal rise
- Liver Biopsy - interface hepatitis, There is periportal piecemeal and bridging necrosis on liver biopsy and cirrhosis
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 screenign for hepatocellular carcinoma