PBC, PSC Flashcards
Primary biliary cirrhosis pathophysiology
Chronic intralobular hepatic bile duct inflammation of small ducts
Primary biliary cirrhosis complications
Cirrhosis and end stage liver disease
40% have inflammatory arthropothies
Primary biliary cirrhosis Presentation
Slow often asymptomatic
Pruritis and Fatigue
Facial pigmentation
Osteoporosis/malacia
Later stage:
- hepatomegaly
- ascites
- jaundice
Primary biliary cirrhosis ratio of females to males
Female: male 9:1
Primary biliary cirrhosis investigations
Abdominal examination Obs Bloods - FBC, LFTs (raised ALP and bilirubin) Serology - AMA antibody Biopsy Abdominal USS
Primary biliary cholangitis Management
Ursodeoxycholic acid
Cholestyramine - Pruritis
If ESLD - transplant
Primary sclerosis cholangitis pathophysiology
Chronic extra + intralobular hepatic bile duct inflammation of the medium to large ducts
Causes chronic biliary obstruction secondary to biliary cirrhosis - causes liver failure
Primary sclerosis cholangitis ratio females to males
2: 1 F>M
Primary sclerosis cholangitis Complications
Associated with inflammatory bowel disease
Cholangiocarcinoma
Primary sclerosis cholangitis Presentation
Often asymptomatic till late stage Fatigue Pruritis Jaundice dark urine, pale stools
Primary sclerosis cholangitis investigations
Abdominal examination Obs Bloods - FBC, U+Es, LFTs (raised ALP + GGT) Serology - pANCA ERCP - gold standard MRCP Urine and stool sample
Primary sclerosis cholangitis management
No cure ore effective intervention
- Liver transplant
Associated condition in primary biliary cholangitis
Sjogrens
RA
Systemic sclerosis
Thyroid disease
