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Medicine year 4 CDM > Block 5: autoimmune liver disease > Flashcards

Block 5: autoimmune liver disease Flashcards

1
Q

Main types and definition of autoimmune liver disease

A

Three main types: Primary Biliary Cholangitis (PBC), Autoimmune hepatitis (AIH), Primary Sclerosing Cholangitis (PSC)

Chronic inflammatory liver disease, rare. Important as it affects young patients and is treatable, can cause significant symptom burden, Range from mild liver disease to cirrhosis

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2
Q

Histological causes of autoimmune liver disease

A
  • Primary biliary cholangitis: classical small bile duct regions, a granulomatous disease
  • Primary sclerosing cholangitis: affects the large bile ducts in the liver, Periductal ‘onionskin’ fibrosis
  • Autoimmune hepatitis: lots of inflammatory cells within the liver, not associated with bile ducts. Disease of the hepatocytes
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3
Q

Primary biliary cholangitis: blood results and demographic

A

Blood results: AMA (anti-mitochondral antibodies) + increased ALP

Demographics
- 90% female, tends to be middle aged women
- Damage to small intrahepatic bile ducts
- Genetic susceptibility and trigger: infectious, environmental toxins, hormones
- Immune insult → Bile duct damage → Inflammation → Fibrosis/Cirrhosis

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4
Q

Symptoms of primary biliary cholangitis

A
  • Pruritus (itching): palms of hand, soles of feet. Worse at night when hot
  • Fatigue
  • Asymptomatic
  • Gastrointestinal symptoms and abdominal pain
  • Jaundice
  • Pale, greasy stools
  • Dark urine
  • Poor memory
  • Dry eyes and mouth
  • Signs of advanced liver disease: jaundice, ascites
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5
Q

Primary biliary cholangitis: on examination

A
  • Xanthoma and xanthelasma(cholesterol deposits)
  • Excoriations(scratches on the skin due to itching)
  • Hepatomegaly
  • Signs of liver cirrhosis and portal hypertension in end-stage disease (e.g., splenomegaly and ascites)
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6
Q

Primary biliary cholangitis: diagnostic criteria

A
  • Abnormal LFTs (cholestatic i.e. raised ALP)
  • Positive anti-mitochondrial antibody (AMA)
  • Compatible histology (liver biopsy): no longer part of routine diagnosis
  • 2 of above is probable PBC, 3 of above is definite PBC
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7
Q

Primary biliary cholangitis: bloods

A
  • Raise alkaline phosphatase(the most notable liver enzyme as with most “obstructive” pathology)
  • Other liver enzymes and bilirubin are raised later in the disease
  • Anti-mitochondrial antibodies(AMA) are the most specific to PBC and form part of the diagnostic criteria
  • Anti-nuclear antibodiesare present in about 35% of patients
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8
Q

PBC treatment: 1

A
  • Ursodeoxycholic acid: its non-toxic, hydrophillic bile acid that protect cholangiocytes from inflammation and damage. Side effects weight gain, hair thinning and diarrhoea. If ALP remains >200 will need extra treatment
  • Fibrates: anti-cholesterol drug, improves LFT’s in PBC, risk of liver injury and renal impairement, Add on therapy
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9
Q

PBC treatment 2

A
  • Obeticholic acid(where UDCA is inadequate or not tolerated – although it can have significant adverse effects). Side effects- itch. New treatment another bile acid. Add on therapy
  • Colestyraminefor symptoms of pruritus (abile acid sequestrantthat reduces intestinal absorption of bile acids)
  • Replacement of fat-soluble vitamins
  • Immunosuppression(e.g., with steroids) is considered in some patients
  • Liver transplantin end-stage liver disease
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10
Q

PBC complications and associations

A
  • Fat-soluble vitamin deficiency (A, D, E and K)
  • Osteoporosis
  • Hyperlipidaemia (raised cholesterol)
  • Sjögren’s syndrome (dry eyes, dry mouth and vaginal dryness)
  • Connective tissue diseases (e.g., systemic sclerosis)
  • Thyroid disease
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11
Q

Pbc investigations

A
  • Ultrasound to exclude biliary obstruction
  • Liver biopsy: usually not required, uncertain diagnosis, possibility of overlap syndrome with autoimmune hepatitis, Florid duct lesion (granuloma + duct obliteration)
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12
Q

PBC itch treatment in order of when you give them

A
  • Bile acid sequestrants (Colestyramine- separated from Ursodeoxycholic acid by 4 hours)
  • Rifampicin: can cause liver injury need blood tests every 2 weeks. Turns body secretions orange i.e. tears
  • Gabapentin
  • Naltrexone → Sertraline → PUVA
  • Dont use antihistamine
  • Can get liver transplant if itch is so bad
  • Iron deficient anaemia can make itch worse
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13
Q

PBC: monitoring

A
  • Review symptoms
  • Osteoporosis: give DEXA scan
  • Monitor for evidence: fibro scan- tests liver stiffness, bloods (low albumin, low platelets, jaundice), ultrasound- portal hypertension, nodular outline
  • If cirrhotic: liver cancer (HCC) screening (ultrasound and AFP) checked every 6 months, varices screening
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14
Q

Shockable rhythms

A

V-fib, V-tach

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15
Q

Narrow complex tachycardia

A
  • Sinus tachycardia (treatment focuses on the underlying cause)
  • Supraventricular tachycardia (treated with vagal manoeuvres and adenosine)
  • Atrial fibrillation (treated with rate control or rhythm control)
  • Atrial flutter (treated with rate control or rhythm control, similar to atrial fibrillation)
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16
Q

Broad complex tachycardia

A
  • Ventricular tachycardia or unclear cause (treated with IV amiodarone)
  • Polymorphic ventricular tachycardia, such as torsades de pointes (treated with IV magnesium)
  • Atrial fibrillation with bundle branch block (treated as AF)
  • Supraventricular tachycardia with bundle branch block (treated as SVT)
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17
Q

Atrial flutter

A
  • Due to a re-entrant rhythm in either atria. Atrial rate 300bpm
  • Long refractory period of AV node means there is two atrial contractions for every one ventricular contraction (2:1 conduction), giving a ventricular rate of 150 beats per minute. There may be 3:1, 4:1 or variable conduction ratios.
  • Saw tooth appearance with narrow complex tachycardia
  • anticoagulation based on the CHA2DS2-VASc score. Radiofrequency ablation of the re-entrant rhythm can be a permanent solution.
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18
Q

Torsades de points

A
  • Type of polymorphic ventricular tachycardia causing twisting along the baseline
  • Will terminate spontaneously or progress to ventricular tachycardia
  • Management: magnesium infusion
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19
Q

Ventricular ectopics

A
  • Premature ventricular beats causes by random electrical discharge. Calls random extra or missed beats
  • More common with pre-existing heart disease
  • Ventricular ectopics appear as isolated, random, abnormal, broad QRS complexes on an otherwise normal ECG.
  • Bigeminy refers to when every other beat is a ventricular ectopic.
  • Mangement: beta blockers
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20
Q

Autoimmune hepatitis

A

A rare cause of chronic hepatitis (inflammation in the liver) it appears to occur due to a combination of genetic and environmental factors.

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21
Q

Type 1 autoimmune hepatitis

A

Typically affects women in their late forties or fifties. It presents around or after menopause with fatigue and features of liver disease on examination. It takes a less acute course than type. Majority of cases, all ages. ANA/ASMA

22
Q

Type 2 autoimmune hepatitis

A

Usually affects children or young people, more commonly girls. It presents with acute hepatitis with high transaminases and jaundice. Liver kidney microsomal (LKM) or liver cytosol antibodies (LC). <10% of cases

23
Q

Autoimmune hepatitis epidemiology

A
  • Immune attack on hepatocytes
  • Affects any age: peaks in children and young adults and 60-70 year olds
  • 70% female
  • Can be precipitated by drugs: Nitrofurantoin, Ritorvastatin
  • Genetic susceptibility + trigger (drug, infectious, environment)
24
Q

Autoantibodies in autoimmune hepatitis

A

Autoantibodiesintype 1 autoimmune hepatitisare:
- Anti-nuclear antibodies (ANA)
- Anti-smooth muscle antibodies (anti-actin)
- Anti-soluble liver antigen (anti-SLA/LP)

Autoantibodiesintype 2 autoimmune hepatitisare:
- Anti-liver kidney microsomes-1 (anti-LKM1)
- Anti-liver cytosol antigen type 1 (anti-LC1)

25
Q

Investigations for autoimmune hepatitis

A

Will show high transaminases (ALT and AST) and minimal change in ALP levels. Raised immunoglobulin G (IgG) levels are an important finding. Liver biopsy forms part of the diagnosis, findings are interface hepatitis and plasma cell infiltration. Will have high ANA and ASMA Likely to have high family history of autoimmune disease

26
Q

Examination, findings and definitions for autoimmune hepatitis

A

Examination: not jaundices, no encephalopathies, no stigmata of chronic liver disease

Findings: ↑ ALT + ANA / ASMA + ↑ IgG

Definition: immune attack on hepatocytes, any age, 70% female. Can be precipitated by drugs. Genetic susceptibility + trigger.

27
Q

Management of autoimmune hepatitis

A
  • Treatmentis withhigh-dose steroids(e.g.,prednisolone). Other immunosuppressants are also used, particularly azathioprine. Immunosuppressant treatment is usually successful at inducing remission (controlling the disease).
  • Liver transplant may be required inend-stage liver disease. Autoimmune hepatitis can reoccur in the new liver.
28
Q

Presentation of autoimmune hepatitis

A
  • Often asymptomatic
  • Symptomatic: Fatigue, anorexia, nausea, joint pains
  • Acute hepatitis: very high ALT
  • Jaundice
  • Complications of cirrhosis
29
Q

Investigations of autoimmune hepatitis

A
  • Combination of: Hepatic LFT’s (high ALT), positive autoantibodies (ANA or SMA), raised immunoglobulins (IgG)- might need to take patient off anticoagulation
  • Liver biopsy: confirm diagnosis, stage fibrosis
  • ↑ ALT + ANA / ASMA + ↑ IgG
30
Q

Treatment of autoimmune hepatitis

A
  • Aim for normal ALT and IgG
  • Immunosuppression: usually lifelong, steroids (prednisolone or budesonide initially), Azathioprone (for maintenance- risk of skin cancer and solid organ skin cancer), Mycophenolate mofetil, Tacrolimus
  • Liver transplantation
31
Q

Risk factors for primary sclerosing cholangitis

A
  • Male
  • 30-40
  • Ulcerative colitis: strong association
  • Family history
32
Q

Presentation of primary sclerosing cholangitis

A
  • Abdominal painin theright upper quadrant
  • Pruritus (itching)
  • Fatigue
  • Jaundice
  • Hepatomegaly
  • Splenomegaly
33
Q

Investigations for PSC

A
  • LFT: raised ALP, other liver enzymes and bilirubin are raised later in the disease
  • Ultrasound and CT often normal: can show portal hypertension
  • Colonoscopyshould be performed to assess for ulcerative colitis.
  • Liver biopsyis not usually required but may be used where there is diagnostic uncertainty. Shows onion skin fibrosis around bile ducts. Use biopsy in small duct PSC
  • ERCP: not used for diagnosis
34
Q

PSC: MRCP

A

It involves an MRI scan that gives a detailed view of the bile ducts, showingbile ductstricturesin primary sclerosing cholangitis. Shows multifocal strictures and segmental dilation. Involvement of intra and extra hepatic ducts. ‘string of beads’ appearance no mass lesion

35
Q

Management of disease in PSC

A
  • Endoscopic retrograde cholangio-pancreatography (ERCP): can be used to treat dominant strictures. Use an endoscope, strictures can be dilated. Antibiotics are given alongside ERCP
  • Replace fat soluble vitamins
  • Dominant stricture: if causing symptoms i.e. cholangitis or itch then treat with ERCP or surgical bypass
  • Liver transplant can be used in advanced disease
36
Q

Management of PSC: symptoms, screening

A
  • Colestyramine for symptoms ofpruritus(abile acid sequestrantthat reduces intestinal absorption of bile acids)
  • Bone disease: DEXA
  • Screen for malignancy: cholangiocarcinoma or colorectal, every 6 months
  • Look for IBD: colonoscopy, if IBD confirmed need annual colonoscopy otherwise every 5 years
  • Monitoring for complications such ascholangiocarcinoma,cirrhosis and oesophageal varices
  • Treat cholangitis, jaundice, itch (same as PBC)
37
Q

Complications and bloods for PSC

A
  • Biliary strictures
  • Acute bacterial cholangitis
  • Cholangiocarcinoma develops in 10-20% of cases
  • Cirrhosis and the related complications (e.g., portal hypertension and oesophageal varices)
  • Fat-soluble vitamin deficiency (A, D, E and K)
  • Osteoporosis
  • Colorectal cancer in patients with ulcerative colitis

Bloods: ANCA + raised ALP

38
Q

What does PSC affect

A
  • Inflammation and fibrosis of intra and extra-hepatic bile ducts causing stricturing
  • Multifocal bile duct strictures
  • Small duct PSC: clinical, biochemical and histological features of PSC but normal cholangiogram (MRCP)
  • 70-80% patients with PSC have inflammatory bowel disease (IBD): normally UC
39
Q

Presentation of PSC

A
  • Often asymptomatic
  • Incidental finding of abnormal LFTs
  • Symptomatic: Fatigue, itch, RUQ pain, weight loss
  • Cholangitis (often not at presentation): Jaundice, abdominal pain, fevers
  • Jaundice and complications of cirrhosis
40
Q

IgG4 related sclerosing cholangitis

A

Similar to primary sclerosing cholangitis (biochemical and MRCP).Elevated IgG4 levelsin the blood are the distinguishing feature. Unlike primary sclerosing cholangitis, IgG4-related sclerosing cholangitis responds well to treatment withsteroids. It is associated withautoimmune pancreatitis. Often extra-hepatic ducts. Can involve pancreas and other organs

41
Q

Overlap syndrome

A
  • Rare: combination of 2 autoimmune liver disease
  • PBC-AIH overlap
  • PSC-AIH overlap
42
Q

Autoantibodies and immunoglobulins

A
  • AMA = Primary biliary cholangitis
  • ANCA/ASMA: Autoimmune hepatitis
  • ANCA = Primary sclerosing cholangitis
  • IgA = Alcohol, NAFLD
  • IgM = Primary biliary cholangitis
  • IgG = Autoimmune hepatitis
43
Q

NAFLD

A

Non-alcoholic fatty liver disease(NAFLD) is characterised by excessive fat in the liver cells, specifically triglycerides. These fat deposits interfere with the functioning of the liver cells. The early stages of NAFLD can be asymptomatic. However, it can progress tohepatitisandliver cirrhosis.

44
Q

What is NAFLD

A
  • Fatty infiltration of the liver in the absence of a secondary cause >5% steatotic hepatocytes
  • Most common liver disease worldwide
  • Liver manifestation of obesity and metabolic syndrome
  • Hepatic steatosis → Steatohepatitis +/- fibrosis → Cirrhosis. Can have regression of fibrosis
45
Q

NAFLD pathogenesis

A

Visceral obesity causes insulin resistance. Increased lipolysis in peripheral fat that increases flow of fatty acids into the liver. Increased lipogenesis and reduced export of fat from the liver Resulting in increased fat in the liver. Can cause inflammation

46
Q

NAFLD symptoms

A
  • Asymptomatic
  • Non specific: fatigue, malaise, RUQ pain
47
Q

NAFLD risk factors

A
  • Middle age onwards
  • Obesity
  • Poor diet and low activity levels
  • Type 2 diabetes
  • High cholesterol
  • High blood pressure
  • Smoking
  • Genetics (PNPLA3, TMSF6 etc)
  • Sleep apnoea
48
Q

NAFLD conservative management

A
  • Weight loss
  • Exercise (all types, independent of weight loss)
  • Mediterranean diet
  • Good diabetes control
  • Coffee consumption
49
Q

NAFLD investigations

A
  • Raised ALT
  • Liver ultrasound: confirms diagnosis of hepatic steatosis, shows increased echogenicity. May not show mild steatosis
  • Liver biopsymay be required to confirm the diagnosis and exclude other causes of liver disease.
  • Transient elastography(“FibroScan”) can be used to assess the stiffness of the liver using high-frequency sound waves. It helps determine the degree offibrosis(scarring) to test forlivercirrhosis. It is used where theenhanced liver fibrosis(ELF) test indicatesadvanced fibrosis.
  • Controlled attenuation parameter (CAP): assessed by fibroscan, Grades steatosis and fatty change
50
Q

Scoring for NAFLD

A
  • NAFLD Fibrosis Score(NFS) is another option for assessing liver fibrosis in NAFLD. It is based on an algorithm of age, BMI, liver enzymes (AST and ALT), platelet count, albumin and diabetes.
  • Fibrosis 4(FIB-4) score is another optionfor assessing liver fibrosis in NAFLD. It is based on an algorithm of age, liver enzymes (AST and ALT) and platelet count. <1.3 excludes advanced fibrosis, lots of false positives
  • Enhanced liver fibrosis (ELF) blood test measures HA, PIIINP and TIMP-1. If 10.51 or above its advanced fibrosis. If under 10.51 its unlikely advanced fibrosis

Medicine year 4 CDM (44 decks)

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