Immune mediated diseases of the liver Flashcards
What are the autoimmune diseases of the bile duct
- primary biliary cholangitis
- primary sclerosing cholangitis
What are the autoimmune diseases of the liver
- autoimmune hepatits
what are the symptoms of primary biliary cholangitis (PBC)
- Often asymptomatic and diagnosed after incidental finding of raised ALP
- jaundice
- sleepiness
- pruritus
- lethargy
Who tend to have primary biliary cholangitis (PBC)
- more common in women 9;1 to men
- usually aged over 40
What is primary biliary cholangitis (PBC)
this is when interlobular ducts are damaged by chronic autoimmune granulomatous inflammation causing cholestasis which may lead to fibrosis, cirrhosis and portal hypertension
What markers increase in primary biliary cholangitis (PBC)
- increase in ALP
- increase in AMA (M2) - antibody presence used as diagnosis
What investigations do you use to show the presence of primary biliary cholangitis
- Bloods
- Ultrasound
- Fibroscan - see how much scarring is in the liver to check for fibrosis and cirrhosis
- Biospy
What is the treatment for primary biliary cholangitis (PBC)
- Ursodeoxycholic acid (UDCA) - 1st line
- Obeticholic acid - for people that dont respond to UDCA
- bezafibrate
- liver transplantation
for symtpoms
- itch - cholestyramine, rifampicin
- bones - risk of osteoporosis - bisphopshonates
- diarrhoea - codeïne phosphate e.g. 30mg/8hour
What is primary sclerosing cholangitis (PSC) symptoms
- jaundice
- pruritus
- cholangitis
- can be asymptomatic
Who does primary sclerosing cholangitis (PSC) effect
- more males than females
- age 20 onwards
- 65% have IBD usually UC
- HLA-A1, B8, DR3
- AIH
What happens in primary sclerosing cholangitis (PSC)
- progressive cholestasis and Large bile duct sclerosis and loss
what cancers are you at risk of developing when you have primary sclerosing cholangitis (PSC)
- cholangiocarcinoma
- colorectal cancer
- hepatobillary cancer
- pancreatic cancer
What is the treatment of primary sclerosing cholangitis (PSC)
No effective treatment
- UDCA - not as effective
- ERCP - stricture treatment
- liver transplantation - mainstay for end stage disease
What is the role of ERCP for primary sclerosing cholangitis (PSC)
- used for dominant strictures - stricture in the main bile duct that is causing blockage of bile flow
- treated with balloon dilatation or stenting or both
- to diagnose cholangiocarcinoma
How does autoimmune hepatitis effect
females more than males 1:3
What is autoimmune hepatitis associated with
- Autoimmune thyoid
- coeliac
- SLE
- vitiligo
- addisons
How do you diagnose autoimmune hepatitis
- High ALT
autoantibodies
- smooth muscle
- ANA
- LKM
- ALSA
- IgG is raised
- liver biopsy
How do you treat autoimmune hepatitis
High dose steroids
- Prednisolone 30mg/d PO for 1 month; ↓ by 5mg/month to a maintenance dose of 5-10mg/d PO
- Can sometimes be stopped after 2yrs but relapse occurs in 50-86%
Immunomodulators
- Azathioprine - 50-100mg/d PO – as steroid-sparing agent to maintain remission
failure of first line therapy
- mycophenolate/ tacrolimus/rituximab
- Liver transplantation
How do you assess the treatment response to autoimmune hepatitis
- ALT
- IgG
- liver biopsy
What is autoimmune hepatitis
- inflammatory liver disease of unknown cause characterised by abnormal T cell function and autoantibodies directed against hepatocytes surface antigens
What are the signs of autoimmune hepatitis
- fever
- malaise
- urticarial rash
- polyarthrtiis
- pleurisy
- pulmonary infiltration
- glomerulonephritis
What are the two types of autoimmune hepatitis
- type 1
- type 2
Describe type 1 autoimmune hepatitis
- seen in 80%
- typical patient is female and under 40 years old
- ASMA +ve in 80%
- ANA +ve in 10%
- good response to immunosuppression in 80%
- 25% have cirrhosis at presentation
Describe type 2 autoimmune hepatitis
- more common in Europe than USA
- more often seen in children
- more commonly progresses to cirrhosis
- less treatable
- typically LKM1 +ve
- ASMA and ANA -ve
