Autoimmune and metabolic liver disease Flashcards
Diagnosis of AIH
F>M ANA positive SMA positive Anti-LKM1 Anti-LKM3 High IgG levels Consistant histology - interface hepatitis extending into the lobule causing hepatic necrosis with rosetting of liver cells
Must exclude other causes of acute hepatitis including drugs
Treatment of AIH
Prednisone and azathioprine
Refractory = calcineurin inhibitors
Primary biliary cirrhosis diagnosis
Middle aged females Fatigue and pruritus AMA and ANA positive High IgM High lipids If AMA negative look for anti-GP210 and anti-SP100
PBC treatment
Treatment = urodeoxycholic acid
Decreases progression of disease
Manage pruiritis - cholestyramine, antihistamines and rifampin
PBC complications
OP
ADEK vitamin deficiency
Lipid issues
Other autoimmune disease
Primary sclerosing cholangitis
? cause
Associated with IBD - UC > Crohns
Diagnosis = MRCP - strictures and beading
Assess activity with LFTs - cholestatic GGT and ALP raised
pANCA positive
Increased risk of cancer - cholangiocarcinoma, gallbladder and bowel cancer
IgG 4 Cholangitis
Looks like PSC
Increased ALT, GGT and ALP
Systemic disease:
- Sjorgens
- Retroperitoneal fibrosis
- Autoimmune pancreatitis
- Psuedotumors - IgG 4
Steroid responsive
Non- alcoholic fatty liver disease diagnosis
Diagnosis = Abnormal LFTs in the overweight
Haemchromatosis
Recessive disease
Mutations = C282Y and H63D
Screening test = iron studies - transferrin saturation >45%
Treatment = venesection aim Ferritin 50-100
Genetic testing for 1st degree relatives
Wilsons Disease
Autosomal recessive
Copper overload
Screening test = low serum ceruloplasmin and high urine ceruloplasmin
Diagnosis = liver biopsy
–> liver disease, neuropsych changes, dystonia, chereoathetoid, parkinsonism
Treatment = zinc, penacillamine, trientine
Main risk factor for NAFLD?
Metabolic syndrome
Increased number of features –> increased risk of NAFLD
Main cause of death with NAFLD?
Cardiovascular disease
INCREASES RISK +++++
Complications of haemochromatosis?
Hypogonadism
Hepatocellular carcinoma
Diabetes mellitus
Yersinia gastroenteritis
Disease decreased with haemochromatosis?
Atherosclerosis
Treatment of NAFLD?
Treatment = weight loss
Mediterranean diet may help