Autoimmune liver disease Flashcards
What is a fibrotic liver like?
continuous inflammation of the liver caused by hepatitis B for example can lead to fibrosis - formation of scar tissue within the liver
What does a cirrhotic liver look like?
scar tissue replaces normal, healthy tissue, blocking the flow of blood through the liver and preventing it from working as it should
What causes autoimmune liver disease?
environmental triggers and genetic predisposition which leads to activated / autoreactive T cells = defective immune system
How does autoimmune hepatitis (AIH) present?
any age : 2- 80 75-90% are women Present any which way - acute liver failure - acute hepatitis - asymptomatic - abnormal liver biochem (elevated AST, ALT, bilirubin) - incidental cirrhosis - decompensated cirrhosis
- Elevated serum IgG in almost all active cases (except acute liver failure and LKM1+VE disease)
- Elevated liver autoantibodies
What are 2 key drugs that can induce an AIH like syndrome?
nitrofurantoin
infliximab
What autoantibodies are indicative of AIH?
using ELISA or blot
- ANA+
- SMA+
- LKM1/LC1+
- SLA/LP+
CONSIDER AIH and do a liver biopsy
What drugs are used to treat AIH and what are the aims of the treatment?
steroids (prednisolone) and azathiopurine
complete remission of
- symptoms
- normal LFTs
- normal immunology - IgG levels
- histology: clearance of hepatitis activity
What is the difference between using budesonide compared to prednisolone?
predisolone is a systemic steroid
budesonide = more locally acting steroid to gut and then it goes to the liver = pts tend to prefer it - only used in more mild disease
What happens if there is no response to steroids in AIH?
It probably isnt AIH
What are the 4 reasons for treatment failure in AIH?
1) got them too late - disease too advanced
2) got the wrong diagnosis
3) no treated them right to start with
4) non-compliance with therapy (big reason)
What are the populations of special consideration in terms of AIH?
Pregnancy
- spontaneous remission; relapse post-partum
Elderly
- 30% have cirrhosis
- respond to lower doses of prednisolone
Acute onset, severe presentation
- jaundiced with INR>1.5, onset <26 weeks
- 20% mortality, 60% need transplant
- treatment only after discussing with a liver transplant unit
Decompensated disease
- benefits vs risk of immunosuppresive treatment
- liaise with liver transplant unit
What are the causes of non-compliance with medication?
limits comms between clinician and pt
developmental issues in adolescence
limited trust by the patient and society for health care providers and health care systems
complexity of the health care system
unhappiness with or lack of knowledge regarding med adverse effects
cost of treatment
complications of comorbid conditions
What are some important features of AIH in young patients <30?
25% +ve for anti-LKM1, often steroid dependent with normal IgG
50% diagnosed below age of 16 develop evolving features characteristic of PSC
azathiopurine and steroids are safe in pregnancy
When should budesonide be considered?
only in non-cirrhotic pts / without portal hypertension
young patietns
elderly than are non-cirrhotic
those likely to experience side effects of systemic steroids (osteoporosis, diabetes)
What is the classical phenotype of Primary biliary cholangitis (PBC)?
90% women 75% women above 50 itching and fatigue high cholesterol often have history of smoking
How is PBC diagnosed?
chronically elevated serum ALP
exclusion of large bile duct obstruction e.g. stone in bile duct, PSC
+ve ANTI-mitochondrail antibody (AMA) in 95%
other autoantibodies e.g. anti-sp100, anti-gp210
raised IgM
liver biopsy rarely needed
What are the treatments for PBC?
treat all pts with elevated ALP
MEDs
- Ursodeoxycholuc acid (UDCA) at 13-15mg/kg/d
goal= attain biochemical response
What does a non-response to meds for PBC indicate?
predicts liver cancer risk
What does obeticholic acid monotherapy improve in PBC?
Significantly improves serum ALP - thought FDA states there are safety concerns with it for rare chronic liver disease
How is the pruritus treated in PBC?
1ST LINE: colestyramine 2nd line - rifampicin caution: - multiple interactions - 20-30% can experience deteriorating liver enzymes (monitor 2-4 weekly) vit K supplementation
Which patients with PBC do badly on treatment?
biochemical non-responders
elevated AST / platelet ratio index
What is PSC?
primary sclerosing cholangitis
- unknown aetiology
- no known treatments
- predominantly affects young people <50
- non-smokers
- non-drinkers
How bad can PSC get?
chronic liver failure
cholangiocarcinoma- most often occurs soon after PSC diagnosis
gallbladder cancer
When should you have a clinical suspicion of CCA in PSC ?
acutely symptomatic presentation
persistently elevated bilirubin
ERCP and brush cytology (accuracy poor)
What disease is PSC commonly associated with?
UC
the prevalence of UC/PSC is greater than the prevalence of UC alone
What is a risk factor for liver transplant failure?
ileal pouch anal anastomosis
What does IBD confer an increased risk of post liver transplant?
hepatic artery thrombosis
What is the current standard of care for PSC?
Cancer surveillance
- annual colonoscopy
- serial cholangiography
- annual ultrasound
Symptom control
- antibiotics for flares of acute cholangitis
- pruritus
- fatigue - ensure vit D replete, TFTs. HbA1c
Complications
- DEXA scanning in those with elevated bilirubin/cirrhosis
- abx in pts with recurrent acute cholangitis
timely assessment for liver transplantation
