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
