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Gastro > MAFLD, ETOH, autoimmune RPA > Flashcards

MAFLD, ETOH, autoimmune RPA Flashcards

1
Q

MAFLD comprises of

A

NAFLD (simple steatosis)

NAFL (ballooning degeneration +/- fibrosis)

NASH (inflammation)

Cirrhosis

Cryptogenic cirrhosis

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2
Q

MAFLD is associated with

A

Obesity
T2DM
Dyslipidaemia
Metabolic syndrome

70-90% in obese or T2DM have MAFLD
20-30% general population

Alternative causes
ETOH
Genetic causes - PNPLA3
Nutritional causes

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3
Q

What is Lean NAFLD?

A 
NAFLD in the absence of obesity
20% Europeans, Americans
Associated with increased visceral obesity, high fructose and fat intake and genetic risk factors
Sedentary
Insulin resistant
High plasma TG levels 
Increased CV risk
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4
Q

Investigations of suspected MAFLD

A

Suspect in those with…
Obesity/metabolic syndrome
Abnormal LFTs (most will be normal) or changes in USS findings

Do...
LFTs
Fasting lipids
Albumin FBC - platelet
Hep serology
ANA
HbA1c 
Liver USS
BP measurement
BMI, waist circumference

Consider…
Abdo CT, USS
Liver USS
Exclude other diseases (not everyone needs a full liver screen)

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5
Q

Risk factor for fibrosis progression in MAFLD

A 
Old age >50
High BMI >28
Rapid weight gain >5kg 
Necroinflammaatory activity (biopsy) 
ALT >2x ULN (and rising)
AST/ALT >1
Elevated TG
Insulin resistance
DM
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6
Q

Tools to assess fibrosis in MAFLD

A

MAFLD fibrosis score

  • Age, BMI, hyperglycaemia, platelet, albumin, AST/ALT ratio
  • Good for ruling in and out but grey zone

Enhanced liver fibrosis panel

Fibroscan

  • Good for excluding fibrosis or correlating with fibrosis if high score
  • High failure rate if high BMI
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7
Q

What do most MAFLD die from?

A

More likely to die from CV disease than liver disease

Hence even if they have zero fibrosis, still important to manage metabolic risk factors

Also increases risk of malignancy including breast ca, HCC, colorectal ca

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8
Q

Management MAFLD

A

Diet

  • Mediterranean diet has positive benefits
  • Composition of diet is important not just weight loss
  • Coffee is beneficial

Exercise

Target insulin resistance

Weight loss aim for 7-10%

Bariatric surgery

  • best evidence due to biggest weight loss
  • CI: portal HTN, cirrhosis

Vitamin E in non-diabetic, non-cirrhotic NASH (not used much)

Metformin and statin may be beneficial

No therapeutic drugs are approved at the moment (lots in trial, published)

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9
Q

Hyperferritinaemia in MAFLD

A

High ferritin very common in NASH
Associated with fibrosis severity and increased all cause mortality
Increased ferritin stores in liver is uncommon

PHELOBOTOMY IS NOT RECOMMENDED

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10
Q

ETOH in MAFLD

A

Don’t consume heavy amounts

But no recommendation re complete abstinence or light ETOH

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11
Q

Liver transplant in MAFLD - how common is it?

A

Currently number 2 indication in Aust/USA

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12
Q

Liver transplant in MAFLD - what other procedure should we consider at the same time?

A

Concomitant sleeve gastrectomy during transplant or after

If not, NAFLD can recur in the graft

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13
Q

What’s a tool used in excess ETOH screening?

A

AUDIT tools

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14
Q

Do all heavy drinkers develop alcoholic liver disease?

A

No

Genetic factors involved

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15
Q

Risk factors for development and disease progression in alcoholic liver disease?

A

Amount and duration of ETOH
Women
Genetic factors

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16
Q

Diagnostic tests for alcoholic liver disease

A 
Indirect markers
GGT
ALT
AST
MCV
Direct markers
EtG
EtS
PEth - new favourite, marker on red cells of ETOH exposure (similar to HbA1c)
FAEEs
17
Q

Evaluation of severity in alcoholic hepatitis

A

Maddrey DF score

- >=32 severe

18
Q

Rx alcoholic hepatitis

A

Prednisolone +/- NAC

improvement, just stop the steroids.

19
Q

Criteria for liver transplant in alcoholic liver disease

A

> 6/12 alcoholic abstiencne
Adequate social support
Review by psychiatrist and drug and EOH team
Concern in patients with active smoking, personality disorder, psychosis, history of ETOH dependence

Some Australian centres are offering liver transplant in 1st alcoholic hepatitis

20
Q

Clinical features autoimmune hepatitis

A 
Elevated AST, ALT
Often jaundice, anorexia, fatigue
Female, bimodal age (young and old)
Hypergammaglobulinaemia
Ciruclating autoantibdies
HLADR3 or DR4
Negative viral markers
1/3 adults have cirrhosis at presentation

Need liver biopsy - ‘typical’ findings

21
Q

Rx autoimmune hepatitis

A

Prednisolone +/- azathioprine

Induction therapy (40-60mg pred +/- azathioprine) followed by maintenance therapy once clinical and biochemical remission is achieved

Very high chance of relapse after stopping treatment
May require life-long low dose therapy

Aim normal AST, ALT and IgG levels

Treatment failure. Consider adding
Cyclosporin
Tacrolimus
6-MP
Mycophenolate mofetil (most experience, particularly those that don't tolerate AZA)
22
Q

Prognosis of autoimmune hepatitis

A

90% mortality at 10 years without treatment

If treated, good prognosis at 10 years even if there is cirrhosis

Needs ongoing monitoring lifelong

23
Q

PBC clinical features

A

95% women
Cholestatic disorder
Positive AMA or ANA

Most diagnosed are asymptomatic now
Typical histology findings but biopsy not done that often

24
Q

Clinical diagnosis of PBC

A

Elevated ALP + AMA at a titre >1:40 is diagnostic

Titre does not correlate with severity, stage or progression

25
Q

AMA positive with normal LFTs

What does it mean?

A

Predicts the eventual development of PBC

Wait until abnormal LFTs before treatment

26
Q

Rx PBC

A

Ursodeoxycholic acid (UDCA)

  • Improves biochemical parameters, reduces death or LTx, delays progression in early stage PBC
  • Put them on this ASAP
  • The earlier you treat the better, especially when there is a biochemical response

Pruritis is a big problem

  • Cholestyramine, rifampicin
  • Fibrates is getting more data now
27
Q

AIH/PBC overlap syndrome

Rx

A

Determine which is the dominant issue
May need biopsy
Need immunosuppression

28
Q

PSC clinical features

A

Chronic, slowly progressive
Rare
Males
Age 30-40

29
Q

PSC is associated with …

A

IBD

Cholangiocarcinoma, HCC and colorectal cancer

30
Q

Prognosis PSC

A

Poor

Cirrhosis is common 1-2 decades post Dx

31
Q

PSC and IBD (especially UC)

What screening do we need to do?

A

Increased risk of colorectal ca

Need annual colonoscopy screening even after liver transplant

32
Q

How to diagnose PSC?

A

MRCP for diagnosis - see typical biliary strictures in the extra and intrahepatic bile ducts

ANCA positive

Biopsy is not usually indicated. Unless diagnosis is uncertain.

33
Q 
PSC
Worsening cholestasis
Weight loss
Raised serum Ca19-9
New or progressive dominant stricture

What are we worried about?

A

Cholangiocarcinoma

34
Q

Management of PSC

A

No medical therapy available

Currently just symptomatic treatment available

  • Treat itch, recurrent bacterial cholangitis
  • Treat dominant strictures (dilatation > strictures)
  • Treat portal HTN

Urseodeoxycholic acid low dose may have some disease modifying effect, but high dose may make things worse

Majority will require transplant(s) but up to 20% will have recurrent PSC

35
Q

IgG4 related systemic disease
Who does it affect?
What conditions is it associated with?

A

Increased serum IgG4
Biopsy evidence of IgG4 producing plasma cells
Elderly men mostly

Associated with autoimmune pancreatitis (most common), Sjogrens syndrome, tubulointerstitial nephritis, retroperitoneal fibrosis, autoimmune hepatitis, autoimmune cholangitis resembling PSC

36
Q

IgG4 related systemic disease Rx

A

Highly responsive to Steroids

Gastro (9 decks)

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