26. Primary biliary cirrhosis. Secondary biliary cirrhosis

Question 1

what is primary billary cholangitis ?

Answer 1

it was previously known as primary billary cirrhosis
autoimmune cholestatic liver disease
histological eveidence by non purulent , grnaulomatous SMALL and medium sized INTRAHEPATIC BILLARY DUCTS affecting inflammtion

90 PERCENT IS FEMALE

Question 2

increased risk factor for primary billary cholangitis ?

Answer 2

familail

association with other autoimmune disease :
rheumatoid arthritis
hashimoto thyroiditis
sojren , sle

Question 3

what are the symptoms of primary billary cholangitis ?

Answer 3

early stage = PRURITIS
arthralgia
HEAVY FATIGUE
RUQ pain and discomfort

dark urine
steatorrhea

severe = jaundice

later - cirrhosisi and cholestaisis = jaundice

Question 4

what are the major complications in PBC ?

Answer 4

fat metabolism disturbnace = hypercholesterolemia
xanthelasma

sicca symptoms / sjoren syndrome = dry eyes / dry mouth

fat solule vit def

recurrent UTI

osteoperosis

Question 5

how can we diagnose Primary billary cholangitis ?

Answer 5

2 out of 3

1) chronically cholestatic parameter : serum alkaline phosphatase (ALP) > 1.5x the upper limit of normal for longer than 6 months - first to appear , increase GGT and conjugated bilirubin

2) AMA postive
or
Primary billary cholangits specific ANA

3) typical histology :LUDWIG AND SCHEUR SYSTEM
stage 1 - normal triad
portal inflammtion
inflammation and slight destruction of billary dcts

stage 2 = enlarged triads
peicemneal necrosis , and periportal fibrosis
proliferation of billary ducts
periductal epitheloid granulomas detected

stage 3 =fibrous septa/ bridge fibrosus
cholestasis , ductopenia
biledrops withn hepatocytes

stage 4= billary cirrhosis and nodules present , lack of bile ducts

mrcp and ercp = only affecting the small and intrahepatic

INTRAHEPATIC SMALL AND MEDIUM SIZED BLE DUCTS AFFECTED

Question 6

treatment ?

Answer 6

UDCA
reduces cholestaisis but does not prevent disease progression
IV antibiotics to treat bacterial infections in ascending cholangitis= ciproflaxin

pruritus = cholestyramine

ERCP - dilate and stent strictures

PSC are often deficient in fat-soluble vitamins (vitamin A, vitamin D, vitamin E, and vitamin K)

liver transplant good prognosis

Question 7

what is secondary billarY cirrhosis ?

Answer 7

it is the long term extrahepatic bile duct obstruction

due to BILLARY ATRESIA , CHOLANGIOCANOMA , CANCER AT THE HEAD OF THE PANCREAS , EXTRAHEPATIC CHOLELITHIASIS

this causes cholestasis of intrahepatic billary tree =

Question 8

diffrences in pbc and psc?

Answer 8

pbc 
intrahepatic small medium bile ducts 
Female mostly 
not really associated with smoking
have dry eyes 
Not a big risk for CHOLANGIOCARINOMA AND COLON CARCINOMA 
No existing inflammtoy bowel disease
ama postive