Lecture 23: Autoimmune/Metabolic Liver Diseases Flashcards
Primary biliary cirrhosis (PBC): gender; pathogenesis
F:M 9:1; susceptibility –> trigger –> immune mediated –> bile duct injury, fibrosis, cirrhosis
PBC affects mostly the…
Bile duct cell
PBC: external triggers
Unknown, maybe env’t (chemicals), or infectious
PBC: dx (tests and name)
Elevated Alk Phos/GGTP, anti-mitochondrial Abs, elevated total serum IgM, “Florid duct lesion”
PBC: histology
Florid duct lesion = inflammation around bile duct –> obliterated bile ducts –> cirrhosis
PBC: presentation
Starts w/ abnormal liver biochemistries, may have pruritis (bile salts under skin), fatigue, and eventually signs/symptoms of liver disease (xanthomas, butterfly hyperpigmentation)
PBC: survival hinges on…
Cirrhosis development (survival drops off)
Autoimmune patients tend to do how with transplants?
Fairly well
Primary sclerosing cholangitis (PSC): gender, pathogenesis, presentation
M:F 3:1, pathogenesis is UNKNOWN (immune perhaps?, infection), elevated cholestatic tests with cholangitis sx (fever, chills, RUQ pain, jaundice)
Are there secondary causes of sclerosing cholangitis?
Yes! due to a variety of causes
PSC: long-term complication
Cholangicarcinoma/cirrhosis
PSC: dx (tests, imaging, and term)
Elevated Alk Phos/GGTP, 75% w/ pANCA, effects intra AND extrahepatic duct sclerosis = ONION SKIN FIBROSIS with strictures in ducts on imaging
pANCA spelled out
Perinuclear anti-neutrophil cytoplasmic antibodies
PSC: histo
Onion skin and loss of bile ducts in triad
Majority of people with PSC have…
IBD
PSC: complications
Cholangitis, end stage liver disease, cholangiocarcinoma (10% risk), higher risk of colon cancer
PSC: tx
Resection, dilate strictures in ducts, medication (ursodeoxycholic acid –> maybe not good), transplant
Autoimmune hepatitis (AiH): gender, age, presentation
F:M 8:1, young and perimenopausal, can be acute hepatitis or insidious
AiH: dx
DIAGNOSIS OF EXCLUSION with some serologies: abnormal serum aminotransferase, high gamma globulin (IgG), and other positive serologies (SMA/ANA), with liver biopsy to rule out other lesions
AiH: tx
Immunosuppression (steroids)
Wilson Disease: inheritance, describe, gene
Autosomal recessive disease of copper metabolism due to ATP7B on chrom 13
What does ATP7B do?
Encodes for a copper transporting ATPase needed to excrete copper in bile and blood
Wilson Disease: pathophys
Accumulation of copper –> hepatocyte death –> Cu accum in brain, kidneys, cornea
Wilson Disease: histo
Rhodamine stain –> Cu deposition