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Flashcards in Autoimmune liver disease Deck (30)
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1
Q

What age does autoimmune hepatitis typically present in

A

40-60 years

2
Q

What HLA types are associated with AIH

A

HLA DR3 = early onset, more severe disease

HLA DR4 = type 1 AIH, later onset, better response to steroids

3
Q

What are the two types of AIH and associated antibodies

A

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
Q

What other immune diseases are associated with each type of AIH

A

Type 1
- thyroiditis, graves, UC, RA

Type 2
- type 1 DM, thyroid disease, vitiligo

5
Q

Clinical presentation of AIH

A

Spectrum from asymptomaric to liver failure

Fatigue, anorexia, itch, nausea, abdo pain

6
Q

Complications of AIH

A

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

7
Q

Diagnosis of AIH

A
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
Q

Treatment of AIH

A

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
Q

What sex is autoimmune hepatitis more common in

A

Female:male
4:1

10
Q

Outcomes in AIH

A

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
Q

Epidemiology of PBC

A

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

12
Q

Pathogenesis of PBC

A

Widely unknown

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

13
Q

Clinical features of PBC

A

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

14
Q

Lab findings in PBC

A
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
Q

Liver biopsy features in PBC

A

Portal and periportal inflammation

Bridging fibrosis and cirrhosis

16
Q

Conditions associated with PBC

A
Sjogrens (40-65%)
Thyroid disease
CREST syndrome
RA
inflammatory arthropathy
17
Q

Criteria for diagnosis of PBC

A

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

Without extra hepatic obstruction or other conditions affecting the liver

18
Q

Treatment of PBC

A

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
Q

Prognosis of PBC

A

Normal life expectancy if diagnosed at early stage

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

20
Q

Epidemiology of PSC

A

Male (70%)
Average age at diagnosis 40 yrs

Associated with UC in 90%

21
Q

Pathology of PSC

A

Cholestatic progressive chronic liver disease

Inflammation and stricturing of intra and extra hepatic bile ducts

22
Q

Genes associated with PSC

A

HLA B8

DRw52a

23
Q

Clinical features of PSC

A

Fatigue, pruritis, RUQ pain, fever with cholangitis

Raised cholestatic liver enzymes and bilirubin
Transaminases less then 300 usually

Associated with pANCA

24
Q

How do you diagnose PSC

A

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
Q

Complications of PSC

A
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
Q

Treatment of PSC

A

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
Q

Screening for malignancy in PSC

A

Annual USS/MRI for gallbladder and cholangio +- ca-19-9

Colonoscopy 1-2 years

28
Q

Clinical features of autoimmune pancreatitis

A

Recurrent pancreatitis
Obstructive jaundice
Strictures
Pancreatic mass

29
Q

Diagnosis of autoimmune pancreatitis

A

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

30
Q

Treatment of autoimmune pancreatitis

A

Glucocorticoids

If co-existing autoimmune cholangitis can use azathioprine