Autoimmune Liver Diseases Flashcards
What is PSC
Primary sclerosing cholangitis
Progressive cholstasis due to bile duct inflammation and strictures
Sclerosing = strictures
Signs and symptoms of PSC
Pruritis
+- fatigue
Advanced: ascending cholangitis, cirrhosis, hepatic failure
Which sex is predominant in PSC and HLA association
Male
HLA-A1, B8, DR3
What other disease is PSC associated with
AIH
IBD-UC
Which combination is associated with malignancy
PSC and
PSC and UC including colorectal malignancy
Other cancers are included risk in PSC
Bile duct
Gal bladder
Liver
Colon
What should be done each year to monitor for malignancy in PSC
US, colonoscopy
Test results show in PSC
Inc ALP inc bilirubin Hypergammaglobunaemia /inc IgM AMA -ve ANA, SMA, ANCA may be +ve ERCP, MRCP - duct damage Biopsy- fibrous, obliterating, cholangitis
Treatment for PSC
Medical Urosdeoxycjolic acid Colestryamine pruritis Antibiotics - bacterial cholangitis Surgical Transplant
What are you still at risk of getting after transplant in PSC
5-10% develop colorectal cancer
What is PBC
Primary biliary cholangitis
Interlobular bile duct damage by chronic autoimmune granulomatous inflammation causing cholestasis
Could also develop fibrosis, cirrhosis, portal hypertension.
Cause of PBC
Unknown
Environmental triggers and generic predisposition
There is a loss of immune tolerance to self mitochondrial proteins
What is the hall mark of PBC
Anti mitochondrial antibodies
Typical age of presentation of PBC
50 years
Risk factors for PBC
FHx
Smoking
Prev pregnancy
Other autoimmune disease
Symptoms and signs of PBC
Asymptotic diagnosed incidentally by inc ALP
may have lethargy, sleepiness and pruritis
Signs- jaundice, skin pigmentation, xanthelasma, xanthomata, hepatosplenomegaly
Complication of PBC
Cirrhosis Osteoporosis Malabsorption of ADEK due to the cholestatsis Osteomalacia - vit d Coagulopathy - vit k HCC
Test results in PBC
Inc ALP Inc gammagt Mild inc AST/ALT Late disease inc bilirubin low albumins Antibody AMA INC IgM USS - exclude extrahepatic cholestasis Biopsy - not usually needed can shoe granulomatois bile duct cirrhosis
Treatment PBC
Medical
Symptomatic
Pruritis - colestyramine, naltrexone, rifampin
Diarrhoea- codiene phosphate
Osteoporosis prevention
Fat soluble vitamin prophylaxis, ADK
Ursodeoxycholic acid inc survival /delay transplant
Obeticholic acid
Monitor LFT USS +- AFP
Surgical
Transplant can get reoccurance 17% in 5 years
PBC prognosis
Variable
Mayo survival model
Predictor
Combine age, bilirubin , albumin, Pt time, oedema, need for diuretics
What is autoimmune hepatitis
It is an inflammatory liver disease of unknown cause
What is the pathogenesis of autoimmune hepatitis
Abnormal T cell function
Autoantibodies against hepatocyte surface antigen
What are the types of autoimmune hepatitis
Type 1 80% of cases female under 40 ASMA anti smooth muscle antibodies +ve 80% ANA antinuclear antibody +ve 10% Inc IgG in 97% 80% respond to immunosuppressant Type 2 Commoner in Europe Children Progresses to cirrhosis Less treatable Anti liver/kidney microsomal type 1 (LKM1) ab +ve ASMA &ANA negative
Complications of autoimmune hepatitis
Complications from cirrhosis and the immunosuppressive drug therapy
How to patients present with autoimmune hepatitis
40% acute hep & signs of autoimmune disease - fever, malaise, urticarial rash, polyarthritis, pleurisy, pulmonary infiltration or glomerulonephritis
60% gradual jaundice or asymptomatic and diagnosed incidental with signs of chronic liver disease
Tests for autoimmune hep
Serum bilirubin, AST ALT ALP inc
Hypergammaglobinaemia
Autoantibodies
Anaemia, dec WCC, dec platelets - hypersplenism
Biopsy: mononuclear infiltrate of portal and peripheral areas & piecemeal necrosis +- fibrosis
Cirrhosis worse prognosis
MRCP to exclude PSC if ALP inc
How to diagnose autoimmune hep
Diagnosis is dependent on excluding other disease
No test is pathognominic
IgG level, auto ab, + histology, in absence of viral disease
Management of auto immune hep
Medical
Immunosuppression - prednisone tapered every month may be in it for 2 years until remission is maintained
Azathioprine can be used as a steroid sparing agent
Can use budensomide instead of prednisone
Surgical liver transplant
Decompensated cirrhosis
Failure of medical treatment
May reoccur
Prognosis of autoimmune hep
Symp or asymptomatic does not make a diff to prognosis
80% 10 year
Less if cirrhosis at presentation
Less if overlap syndromes PSC and UC and autoimmune cholangitis
Associations with autoimmune hep
Pernicious anaemia UC Glomeruloneohritus Autoimmune thyroiditis Autoimmune heamolysis DM PSC HLA A1 B8 DR3
