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Flashcards in Autoimmune liver disease Deck (30):
1

What age does autoimmune hepatitis typically present in

40-60 years

2

What HLA types are associated with AIH

HLA DR3 = early onset, more severe disease
HLA DR4 = type 1 AIH, later onset, better response to steroids

3

What are the two types of AIH and associated antibodies

Type 1 (Classic)
- adolescents and adults
- ANA, ASMA, anti-actin antibodies, anti-SLA/LP, p-anca, AMA

Type 2
- occurs at younger age, generally more severe
- can occur in infants
- anti-LKM-1 ab, anti-LC-1 ab, anti-SLA/LP

4

What other immune diseases are associated with each type of AIH

Type 1
- thyroiditis, graves, UC, RA

Type 2
- type 1 DM, thyroid disease, vitiligo

5

Clinical presentation of AIH

Spectrum from asymptomaric to liver failure
Fatigue, anorexia, itch, nausea, abdo pain

6

Complications of AIH

Cirrhosis, HCC (though lower rates then other causes of cirrhosis) and liver failure

7

Diagnosis of AIH

Abnormal LFTs (aminotransferases higher then cholestatic enzymes)
Elevated serum globulins with a polyclonal (usually IgG) response
Test for range of antibodies
Liver biopsy - interface hepatitis, lymphocytic infiltration in portal tract, hepatic rosette formation

8

Treatment of AIH

Glucocorticoid -60mg of prednisone then taper
Maintenance - azathioprine + lower dose steroid

90% will have improvement in LFTs in 2 weeks
50-90% will relapse after stopping treatment (predicted by histology - normal= lower rates of relapse)

9

What sex is autoimmune hepatitis more common in

Female:male
4:1

10

Outcomes in AIH

30% have cirrhosis at diagnosis, 30-50% develop cirrhosis
5% develop HCC
10-20% need liver transplant
AIH recurs in transplant in 20-30%

10 year survival with treatment 90%

11

Epidemiology of PBC

Rare, 100 per million
Mostly women 90%
Middle age 40-60
Common in Northern Europe and North America

12

Pathogenesis of PBC

Widely unknown
T cell attack on small intra-lobular bile ducts leading to destruction and disappearance

13

Clinical features of PBC

Asymptomatic in 50-60%
Fatigue, puritis
Skin hyperpigmentation due to melanin deposition in 25-50%
Xanthomas, xanthelesma due to high levels of HDL

14

Lab findings in PBC

Elevated cholestatic liver enzymes
Mildly raised transaminases
Bilirubin elevated in late disease
AMA positive in 95%
Commonly raised ANA 70%
High HDL - does not correspond to higher risk of CVD

15

Liver biopsy features in PBC

Portal and periportal inflammation
Bridging fibrosis and cirrhosis

16

Conditions associated with PBC

Sjogrens (40-65%)
Thyroid disease
CREST syndrome
RA
inflammatory arthropathy

17

Criteria for diagnosis of PBC

ALP greater then 1.5 the ULN
AMA positive
Histological evidence of PBC

Without extra hepatic obstruction or other conditions affecting the liver

18

Treatment of PBC

Vitamin supplementation
Restrict dietary fat
Control cholesterol
Consider enzyme replacement

UDCA (ursodeoxycholic acid) reduces progression to end stage disease and increases survival

Liver transplant still used for refractory cases
PBC reoccurs in transplant in 30% after 10 years

19

Prognosis of PBC

Normal life expectancy if diagnosed at early stage

Some progression to cirrhosis, HCC, metabolic bone disease and malabsorption

20

Epidemiology of PSC

Male (70%)
Average age at diagnosis 40 yrs

Associated with UC in 90%

21

Pathology of PSC

Cholestatic progressive chronic liver disease
Inflammation and stricturing of intra and extra hepatic bile ducts

22

Genes associated with PSC

HLA B8
DRw52a

23

Clinical features of PSC

Fatigue, pruritis, RUQ pain, fever with cholangitis

Raised cholestatic liver enzymes and bilirubin
Transaminases less then 300 usually

Associated with pANCA

24

How do you diagnose PSC

Gold standard is cholangiography (MRCP etc.)
Shows characteristic beaded appearance of bile ducts with multifocal strictures

Liver biopsy may support diagnosis but is rarely diagnostic

25

Complications of PSC

Vitamin deficiency (DEKA)
Metabolic bone disease
Biliary strictures
Higher risk of cholangitis and cholelothiasis, highest risk after ERCP
Cirrhosis and portal hypertension
Cancer
- cholangio = 10-15% risk
- gallbladder carcinoma
- HCC
- colorectal ca (independent of UC risk)

26

Treatment of PSC

No treatment slows progression
UDCA can improve symptoms but often get worse symptoms once it is stopped
Dilating dominant strictures
Liver transplant has best outcomes

27

Screening for malignancy in PSC

Annual USS/MRI for gallbladder and cholangio +\- ca-19-9
Colonoscopy 1-2 years

28

Clinical features of autoimmune pancreatitis

Recurrent pancreatitis
Obstructive jaundice
Strictures
Pancreatic mass

29

Diagnosis of autoimmune pancreatitis

IgG4 plasma cell infiltrates
IgG4 greater then 2x the ULN
Imaging = diffuse enlarged pancreas, irregularities of pancreatic and bile ducts

30

Treatment of autoimmune pancreatitis

Glucocorticoids
If co-existing autoimmune cholangitis can use azathioprine