The 6 C’s of Primary Sclerosing Cholangitis (PSC)
PSC is a chronic, cholestatic, immune-mediated disease characterized by progressive inflammation, fibrosis, and destruction of intra- and extra-hepatic bile ducts, commonly leading to multi-focal biliary strictures and eventual progression to end-stage liver disease.
• COVERT - PSC affects both sexes and occurs at all ages; a majority of patients are male and the median age at onset is 30–40 years. More than two-thirds of patients will have concomitant IBD. Approximately 50% of patients with PSC are asymptomatic at diagnosis.
• CHOLANGITIS - Genetic and environmental factors interact to establish the pathogenesis of PSC.
• CIRRHOSIS - Progression to cirrhosis is most frequent in symptomatic patients with persistently active disease biochemically. Whilst there are many indications for liver transplantation in PSC, it is mostly indicated for decompensated cirrhosis.
• COLITIS - PSC-IBD is phenotypically distinct from IBD without PSC
• CANCER - PSC patients are at increased risk of colorectal and hepato-biliary cancers, including cholangiocarcinoma, hepatocellular carcinoma, and gallbladder cancer
• CURE - Care focuses on symptom control, judicious use of ERCP, and end-stage liver disease management. Therapy with UDCA remains common but evidence for efficacy is challenged by ongoing outcome data. Some advocate a trial of UDCA for all patietns, with cessation if insignificant biochemical response. Other approaches remain subject to clinical trial evaluation.
Dr. Gideon Hirschfield @AutoImmuneLiver
#PSC #Primary #Sclerosing #Cholangitis #diagnosis #management #summary #Hepatology
Algorithm for the Diagnosis of Primary Biliary Cholangitis (PBC).
US, ultrasound; MRCP, magnetic resonance cholangiopancreatography; ALP, alkaline phosphatase; GGT, gamma-glutamyl transferase; AMA, anti-mitochondrial antibodies; ANA, anti-nuclear antibodies
#Primary #Biliary #Cholangitis #PBC #Algorithm #Diagnosis #Hepatology
Proposed Schema for the Management of Pruritus in Primary Biliary Cholangitis (PBC).
Cholestyramine up to 4g x 4day
Rifampicin 150 mg/day
Increase up to 60 mg/day every other week
Naltrexone up to 50 mg/day
Sertraline up to 100 mg/day
Extracorporeal albumin dialysis
Plasmapheresis
Consider transplantation
d, day; h, hours; EOW, every other week; MARS, Molecular Adsorbent Recirculating System; CBD, common bile duct
#Pruritus #Primary #Biliary #Cholangitis #PBC #Management #Hepatology #Algorithm
Primary Biliary Cirrhosis (PBC) - Summary
PBC Epidemiology:
• Female:Male 9:1
• Common European descent
• Age: 30-65 years
PBC Pathophysiology:
• T lymphocyte mediated attack on small intralobular bile ducts
• Loss of intralobular bile ducts causes cholestasis → Cirrhosis/Liver failure
PBC Signs and Symptoms:
• 50-60% are asymptomatic
• Pruritus
• Fatigue
PBC Physical Examination:
• Skin hyperpigmentation
• Jaundice (later manifestation)
• Xanthomas
• Hepatosplenomegaly
• Spider nevi
• Temporal/Proximal limb muscle wasting
PBC Labs:
• Alkaline phosphatase
• Gamma-glutamyltransferase (GGT)
• 5-Nucleotidase
• ALT & AST normal or slightly elevated
• Bilirubin - Normal early on but elevated as disease progresses
• +AMA - 95% of patients
• +ANA - up to 70%
• IgM
• Hypercholesterolemia (85%)
• Ceruloplasmin
• Antithyroid antibodies
PBC Diagnosis:
• No extrahepatic biliary obstruction
• ALP > 1.5 x ULN
• AMA titer 1:40 or higher
• Liver biopsy - Histo shows nonsuppurative destructive cholangitis and destruction of interlobular bile ducts
Differential Diagnosis:
• Viral hepatitis, TPN, Intrahepatic cholestasis of pregnancy, Cardiac diseases, Endocrinopathy, Lymphoma and solid organ malignancies, Hepatic amyloidosis, Bacterial, fungal, viral infections, Sarcoidosis, Drug-induced cholestasis, Primary sclerosis group cholangitis, IgG4 related disease, Bile duct obstruction - gallstone or malignancy
#Primary #Biliary #Cirrhosis #PBC #cholangitis #diagnosis #workup #hepatology
