Primary Sclerosing Cholangitis

Diagnosis

Imaging

MRCP preferred over ERCP for diagnosis (noninvasive, less expensive, no risk of pancreatitis).
Strong recommendation
Moderate quality evidence

Biopsy

Liver biopsy not necessary to establish PSC diagnosis if cholangiographic findings are diagnostic.
Conditional recommendation
Low quality evidence
Liver biopsy recommended for suspected small duct PSC or to exclude other conditions (e.g. overlap with AIH).
Conditional recommendation
Moderate quality evidence

Labs

AMA testing can help exclude PBC.
Conditional recommendation
Moderate quality evidence
Should test for elevated serum IgG4 at least once (PSC with elevated IgG4 may have rapidly progressive disease and/or potentially respond to steroids).
Conditional recommendation
Moderate quality evidence

ERCP

ERCP + balloon dilation recommended if dominant stricture (CBD <1.5 mm, hepatic ducts <1.0 mm) and pruritus, and/or cholangitis.
Strong recommendation
Low quality evidence
If dominant stricture on imaging, should do ERCP + cytology, biopsies, and FISH to exclude cholangiocarcinoma.
Strong recommendation
Low quality evidence
Should give prophylactic antibiotics before ERCP to prevent post-ERCP cholangitis.
Conditional recommendation
Low quality evidence
Routine stenting after dilation of dominant stricture not required; short-term stenting may be required if severe stricture.
Conditional recommendation
Low quality evidence

General

If mild pruritus, should treat with skin emollients and/or antihistamines.
Conditional recommendation
Very low quality evidence
If moderate pruritus, should treat with bile acid sequestrants (e.g., cholestyramine 4–16 g/d); if ineffective or not tolerated, can consider rifampin 150–300 mg BID (monitor for hepatotoxicity) or naltrexone (up to 50 mg/d).
Conditional recommendation
Very low quality evidence
Recommend screening for varices if advanced PSC or platelets < 150K × 10³/dL.
Conditional recommendation
Very low quality evidence
At PSC diagnosis, recommend DEXA scan and repeat at 2-4-year intervals.
Conditional recommendation
Moderate quality evidence
If advanced PSC, should monitor for fat-soluble vitamin deficiencies (i.e., A, D, E, K).
Conditional recommendation
Moderate quality evidence

Liver Transplant

Liver transplant recommended (when possible) over medical therapy or surgical drainage in PSC with decompensated cirrhosis (prolongs survival).
Strong recommendation
Moderate quality evidence
If MELD >14, should refer for liver transplant (5-year post-transplant survival 80-85%, but risk of PSC recurrence).
Conditional recommendation
Moderate quality evidence

Medical Treatment

Should not use UDCA in doses >28 mg/kg/d (increased need for transplant, varices, and colonic neoplasia in PSC with UC).
Strong recommendation
High quality evidence

Special Situations

If age <25 or higher-than-expected aminotransferases (5x ULN), recommend testing for AIH (~10% prevalence in PSC).
Conditional recommendation
Moderate quality evidence
If age <25 with AIH and serum ALP > 2x ULN, recommend MRCP to test for PSC.
Conditional recommendation
Moderate quality evidence

HPB Malignancies and Gallbladder Disease

Consider US or MRCP + serial CA 19-9 every 6-12 months to screen for cholangiocarcinoma (10-year cumulative risk 8%).
Conditional recommendation
Very low quality evidence
If GB polyps >8 mm, recommend cholecystectomy to prevent GB adenocarcinoma, and recommend annual US for GB cancer surveillance.
Conditional recommendation
Very low quality evidence

PSC and IBD

In PSC + IBD, recommend annual CRC screening with chromoendoscopy (4-5x increased dysplasia/cancer risk with PSC + IBD as compared to IBD without PSC).
Conditional recommendation
Moderate quality evidence
At time of PSC dx, full colonoscopy + bx recommended to evaluate for IBD.
Conditional recommendation
Moderate quality evidence
If no IBD on colonoscopy, some advocate repeat colonoscopy every 3–5 years.
Weak recommendation
Low quality evidence