Autoimmune hepato-biliary causes Flashcards
In which patient group is autoimmune hepatitis more commonly seen?
young females
What are 3 recognised associations of autoimmune hepatitis?
- Autoimmune disorders
- Hypergammaglobulinaemia
- HLA B8 DR3
What can autoimmune hepatitis be based upon?
types of circulating antibodies present
What are the 3 types of autoimmune hepatitis and the associated circulating antibodies?
- Type I: anti-nuclear antibodies (ANA) and/or anti-smooth muscle antibodies (SMA)
- Type II: anti-liver/kidney microsomal type 1 antibodies (LKM1)
- Type III: soluble liver-kidney antigen
Which age groups does type I autoimmune hepatitis (ANA and SMA) commonly affect?
both adults and children
Which age group does type II autoimmune hepatitis (anti-LKM1) commonly affect?
affects children only
Which age group does type III autoimmune hepatitis (liver-kidney antigen) most commonly affect?
adults in middle-age
What are 8 clinical features of autoimmune hepatitis?
- Jaundice, fever (acute hepatitis signs)
- Signs of chronic liver disease
- Fatigue
- Loss of appetitie
- Hepatomegaly
- Splenomegaly
- Abdominal pain
- Amenorrhoea (common)
What are 4 key investigations for autoimmune hepatitis and what will they show?
- Deranged LFTs: ALT and bilirubin raised, normal/mildly raised ALP
- ANA/SMA/LKM1 antibodies
- IgG predominant hypergammaglobulinaemia
- Liver biopsy: inflammation extending beyond limiting plate (piecemeal necrosis), bridging necrosis
Which is the commonest type of autoimmune hepatitis?
Type 1
What proportion of patients with autoimmune hepatitis Type I will have raised anti-SMA vs raised ANA?
80% anti-SMA, 10% ANA
What are 3 aspects of the management of autoimmune hepatitis?
- Steroids
- Other immunosuppressants e.g. azathioprine
- Liver transplantation
In which patient group is primary biliary cholangitis most commonly seen?
middle-aged females
What is the gender ratio affected by primary biliary cholangitis?
F:M 9:1
What is the pathophysioloy of primary biliary cholangitis?
interlobular bile ducts become damaged by a chronic inflammatory process, leads to scarring, causing progressive cholestasis which may eventually progress to cirrhosis
What can primary biliary cholangitis progress to?
cirrhosis (previously called primary biliary cirrhosis)
What is the classic presentation of of primary biliary cholangitis?
itching in a middle-aged woman
What are 4 associations of primary biliary cholangitis?
- Sjogren’s syndrome (up to 80%)
- rheumatoid arthritis
- systemic sclerosis
- thyroid disease
What proportion of patients with primary biliary cholangitis also have Sjogren’s syndrome?
80%
What are 10 clinical features of primary biliary cholangitis?
- Extreme fatigue
- Itching
- Dry skin
- Dry eyes
- Jaundice
- Hyperpigmentation, especially over pressure points
- Right upper quadrant pain
- Clubbing
- Hepatosplenomegaly
- Xanthelasmata
Which antibody type is highly sensitive and specific for primary biliary cholangitis?
anti-mitochrondial antibodies (AMA) M2 subtype
What are 3 types of antibodies which may be present in PBC and may aid diagnosis?
- Anti-mitochondrial antibody (AMA) type M2 (98%)
- Smooth muscle antibodies (30%)
- Raised serum IgM
What are 4 types of investigations key to making a diagnosis of PBC?
- LFTs - deranged
- Antibodies: Positive AMA (anti-mitochondrial antibodies), SMA, IgM
- Abdomional ultrasound
- Liver biopsy - inflammation and scarring
Wat are 4 aspects of the management of primary biliary cirrhosis?
- First line: ursodeoxycholic acid
- Cholestyramine for pruritus
- Fat-soluble vitamin supplements
- Liver transplantation
What is the first line management of PBC and what is its effect?
Ursodeoxycholic acid: slows disease progression and imrpvoes symptoms
What is an indication for liver transplantation in PBC?
bilirubin >100
What is a problem with liver transplantation in PBC?
recurrence in graft can occur - but not usually a problem
What are 3 complications of PBC?
- Cirrhosis - portal hypertension, ascites, variceal haemorrhage
- Osteomalacia and osteoporosis
- Significantly increased risk of hepatocellular carcinoma (20x risk)
Why does PBC have a poor mortality?
risk of hepatocellular carcinoma
What is primary sclerosing cholangitis?
chronic choelstatic disorder characterised by inflammation and fibrosis of intrahepatic and extrahepatic bile ducts, resulting in multifocal biliary strictures
What are 3 key associatiosn of primary sclerosing cholangitis?
- Ulcerative colitis: 4% of patients with UC have PSC but 80% of patients with PSC have UC
- Crohn’s (less commoner than UC)
- HIV
What proportion of patient’s with PSC have ulcerative colitis?
80%
What are 8 clinical features of primary sclerosing cholangitis?
- Can be asymptomatic with abnormal LFTs
- Hepatomegaly
- Jaundice
- Right upper quadrant pain
- Fatigue
- Weight loss
- Fevers
- Sweats
What will LFTs show in PSC?
raised bilirubin and ALP (cholestatic picture)
What are 6 investigations to perform to diagnose PSC?
- LFTs (cholestatic picture)
- Positive anti-smooth muscle - SMA
- Anti-nuclear antibodies - ANA
- Myeloperoxidase antineutrophil cytoplasmic antibody - p-ANCA
- MRCP: multiple beaded biliary strictures
- ERCP: shows beaded biliary strictures
What are 3 types of antibodies which may be positive for primary sclerosing cholangitis?
- ANA
- SMA
- p-ANCA
What are the standard diagnostic investigations for primary sclerosing cholangitis?
ERCP or MRCP
What may liver biopsy show in PSC and is it indicated?
limited role for it
may should fibrous, obliterative cholangitis often described as ‘onion skin’
What are 2 complications of PSC?
- Cholangiocarcinoma (10%)
- Increased risk of colorectal cancer
What are 5 aspects of the management of PSC?
- Avoid alcohol
- Pruritis can be managed with cholestyramine
- Supplement fat-soluble vitamins (ADEK)
- Strictures can be dilated via ERCP
- Liver transplantation may be indicated in cases complicated by chronic liver disease and/or hepatobiliary malignancies
