Primary Sclerosing Cholangitis (PSC)

Expect ERCP, and expect repeats - PSC is managed endoscopically. A dominant stricture — under 1.5 mm in the common bile duct or under 1.0 mm in the hepatic ducts — with pruritus or cholangitis gets ERCP with balloon dilation, and these patients come back for it. Each anesthetic lands on a liver further along than the last, so pull current labs rather than reusing the old chart.

Check clotting before planning neuraxial - Cholestasis blocks fat-soluble vitamin absorption, and vitamin K deficiency deranges the clotting factors. Get a PT/INR and platelet count. In chronic liver disease the picture is not simply hypocoagulable — the natural anticoagulants are low alongside the factors — so thromboelastography reads the whole clot rather than one arm of it, and TEG-guided therapy reduces the blood products these patients receive.

Read liver reserve off the labs - An elevated alkaline phosphatase is the signature; transaminases run only 2 to 3 times normal. Bilirubin and albumin are often normal at diagnosis, so a rising bilirubin or falling albumin means advanced disease, a dominant stricture, or cirrhosis. A MELD above 14 is the transplant referral threshold, and the Mayo risk score (age, bilirubin, albumin, AST, prior variceal bleeding) predicts survival.

Cholangitis is the emergency - Fever, chills, and right upper quadrant pain with or without jaundice is ascending cholangitis. Charcot's triad is pain, fever, jaundice; Reynolds pentad adds altered mental status and hemodynamic shock. Treat it as biliary sepsis with an undrained source — it does not improve until the duct is decompressed, so resuscitate toward the procedure rather than instead of it.

Give antibiotics before ERCP in obstruction - Prophylaxis is not routine for ERCP, but it is recommended when there is biliary obstruction or a liver transplant. Cover biliary flora — enterococci and gram-negatives — and continue afterward if drainage was incomplete.

Ask when cholestyramine was last taken - The bile acid sequestrant given for pruritus interferes with absorption of many oral drugs, including propranolol, thiazides, warfarin, digoxin, thyroxine, and statins, and it worsens the fat-soluble vitamin deficiency it sits on top of. A home beta blocker or anticoagulant may not be on board the way the med list implies.

Portal hypertension follows the scarring - Biliary fibrosis raises portal pressure, and late disease brings GI bleeding, ascites, and confusion. Prior variceal bleeding carries enough weight to sit in the Mayo risk score — ask about it directly rather than inferring it from the diagnosis.

Move these patients deliberately - Bone density is checked at diagnosis and every 2 to 4 years because osteoporosis is expected; vitamin D and vitamin K malabsorption both feed it. Activity is restricted in advanced osteoporosis because of fracture risk, which is the same reason to pad thoroughly, position slowly, and avoid forced range of motion in a patient who cannot tell you it hurts.

Post-transplant patients are immunosuppressed - Transplant is definitive for decompensated cirrhosis, with 5-year survival up to 80%, but it trades liver failure for immunosuppression and its complications. Strict asepsis for lines and neuraxial, and a lower threshold for treating an infection as real.

Watch for cholangiocarcinoma - Ten to twenty percent of PSC patients develop it, and CA 19-9 is followed as a screen. Weight loss with a new obstructive picture may mean you are anesthetizing for a cancer operation, not a benign stricture.

Primary sclerosing cholangitis (PSC) is a chronic, progressive cholestatic disease in which inflammation, fibrosis, and stricturing scar the intrahepatic and extrahepatic bile ducts. Concentric periductal "onion-skin" fibrosis displaces the peribiliary capillary plexus, leaving the duct ischemic and driving further scarring — a self-feeding loop that ends in cholestasis, parenchymal injury, and portal hypertension as fibrosis compresses the portal triads. Sixty to eighty percent of patients also have inflammatory bowel disease, predominantly ulcerative colitis. Median survival from diagnosis to death without transplant is about 10 years, and 10% to 20% develop cholangiocarcinoma.

Three consequences drive the anesthetic. Obstructed bile flow blocks absorption of the fat-soluble vitamins A, D, E, and K, so the patient may arrive with a vitamin K-dependent coagulopathy and osteoporotic bone. An obstructed duct that infects becomes ascending cholangitis and septic shock. And endoscopic management means repeat anesthetics on a liver that is further along each time.