Autoimmune Liver Diseases

Question 1

What is PSC

Answer 1

Primary sclerosing cholangitis
Progressive cholstasis due to bile duct inflammation and strictures

Sclerosing = strictures

Question 2

Signs and symptoms of PSC

Answer 2

Pruritis
+- fatigue
Advanced: ascending cholangitis, cirrhosis, hepatic failure

Question 3

Which sex is predominant in PSC and HLA association

Answer 3

Male

HLA-A1, B8, DR3

Question 4

What other disease is PSC associated with

Answer 4

AIH

IBD-UC

Question 5

Which combination is associated with malignancy

PSC and

Answer 5

PSC and UC including colorectal malignancy

Question 6

Other cancers are included risk in PSC

Answer 6

Bile duct
Gal bladder
Liver
Colon

Question 7

What should be done each year to monitor for malignancy in PSC

Answer 7

US, colonoscopy

Question 8

Test results show in PSC

Answer 8

Inc ALP
inc bilirubin
Hypergammaglobunaemia /inc IgM
AMA -ve
ANA, SMA, ANCA may be +ve
ERCP, MRCP - duct damage
Biopsy- fibrous, obliterating, cholangitis

Question 9

Treatment for PSC

Answer 9

Medical 
Urosdeoxycjolic acid 
Colestryamine pruritis
Antibiotics - bacterial cholangitis
Surgical 
Transplant

Question 10

What are you still at risk of getting after transplant in PSC

Answer 10

5-10% develop colorectal cancer

Question 11

What is PBC

Answer 11

Primary biliary cholangitis
Interlobular bile duct damage by chronic autoimmune granulomatous inflammation causing cholestasis
Could also develop fibrosis, cirrhosis, portal hypertension.

Question 12

Cause of PBC

Answer 12

Unknown
Environmental triggers and generic predisposition
There is a loss of immune tolerance to self mitochondrial proteins

Question 13

What is the hall mark of PBC

Answer 13

Anti mitochondrial antibodies

Question 14

Typical age of presentation of PBC

Answer 14

50 years

Question 15

Risk factors for PBC

Answer 15

FHx
Smoking
Prev pregnancy
Other autoimmune disease

Question 16

Symptoms and signs of PBC

Answer 16

Asymptotic diagnosed incidentally by inc ALP
may have lethargy, sleepiness and pruritis
Signs- jaundice, skin pigmentation, xanthelasma, xanthomata, hepatosplenomegaly

Question 17

Complication of PBC

Answer 17

Cirrhosis
Osteoporosis
Malabsorption of ADEK due to the cholestatsis
Osteomalacia - vit d
Coagulopathy - vit k 
HCC

Question 18

Test results in PBC

Answer 18

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

Question 19

Treatment PBC

Answer 19

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

Question 20

PBC prognosis

Answer 20

Variable
Mayo survival model
Predictor
Combine age, bilirubin , albumin, Pt time, oedema, need for diuretics

Question 21

What is autoimmune hepatitis

Answer 21

It is an inflammatory liver disease of unknown cause

Question 22

What is the pathogenesis of autoimmune hepatitis

Answer 22

Abnormal T cell function

Autoantibodies against hepatocyte surface antigen

Question 23

What are the types of autoimmune hepatitis

Answer 23

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

Question 24

Complications of autoimmune hepatitis

Answer 24

Complications from cirrhosis and the immunosuppressive drug therapy

Question 25

How to patients present with autoimmune hepatitis

Answer 25

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

Question 26

Tests for autoimmune hep

Answer 26

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

Question 27

How to diagnose autoimmune hep

Answer 27

Diagnosis is dependent on excluding other disease
No test is pathognominic
IgG level, auto ab, + histology, in absence of viral disease

Question 28

Management of auto immune hep

Answer 28

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

Question 29

Prognosis of autoimmune hep

Answer 29

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

Question 30

Associations with autoimmune hep

Answer 30

Pernicious anaemia
UC
Glomeruloneohritus
Autoimmune thyroiditis 
Autoimmune heamolysis
DM
PSC
HLA A1 B8 DR3