PBC and PSC Flashcards
what is thee pathophysiology of PBC ?
autoimmune disease where the small intralobular bile ducts are affected , causes inflammation of these small bile ducts , followed by outflow obstruction causing cholestasis
what is the clinical piicture of PBC ?
middle aged female
jaundiced
complains of pale greasy stools
signs of liver cirrhosis
xanthoma or xanthelasma
fever and rigors do not occur
what are the other diseases associated with PBC ?
autoimmune hashimoto thyroiditis
celiac disease
sjogren syndrome
systeemic sclerosis
IBD
what are the autoantibodies associated with PBC ?
AMA positive - more specific to PBC
ANA positive and anti smooth muscle in some patients may be positive
what is thee serum testing result that indicates biliary obstruction ?
high ALP
high GGT
conjugated hyperbilirubinemia in the more advanced stages
what is the serum testing resultt for PBC ?
High ALP
High AMA
what are the different sources of raised ALP ?
rapid bone growth in children
pregnancy
pagets disease
vitamin d deficiency
how do we know if elevated ALP is due to liver affection ?
if the elevated ALP is accompanied by elevated GGT or high direct bilirubin
what is the first line investigation for the diagnosis of PBC ?
abdominal ultrasound in order to exclude causes of mechanical obstruction
wheen is liver biopsy indicated in PBC ?
if there is uncertainty in the diagnosis
what is the management for PBC ?
UDCA as the first line therapy ( for life)
Cholestyramine
what are some other issues associated with PBC ?
steatorrhea
osteoporosis
HCC
how should a patient with osteoporosis due to PBC be managed ?
regular DEXA scan at prresntation
vitamin DD supplementation
bisphophonates
what must be taken into consideration for patients taking bisphophonates and have PBC ?
must be carefully used for patients with varicies
when should liver transplant be taken into consideration in cases of PBC ?
with liver cirrhosis
what is the pathogenesis with PSC ?
progressive fibrosis and destruction of the intra and extrahepatic ducts of any and all sizes
what are the diagnostic criteria for PSC ?
generalized beading and stenosis of the biliary system
abscence of cholledocholithiasis
exclusion of bile duct cancer
what is the C/P of PSC ?
middle aged male
fatigue
intermittent jaundice
RUQ pain
Pruritus
what other conditions is PSC associated with ?
Ulcerative colitis
what is the most common cause of death is PSC ?
cholangiocarcinoma
what is a cholestatic pattern in lab tests ?
disproportionate rise in ALP in comparison to ALT and AST
what is the key investigation for PSC ?
MRCP
what is the diagnostic finding on liver biopsy showing early features of PSC ?
periductal onion skin
later on becomes vanishing duct syndrome
what is the trreatment for PSC ?
no definitive treatment
manage complications
cholestyramine is the first line treatment UDCA should not be used
ERCP biliary dilatation
what are the antibodies found in PSC ?
p-ANCA
ANA
anticardiolipin antibodies
