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    Primary Sclerosing Cholangitis (beta)

    Official guideline from the American College of Gastroenterology

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    Diagnosis

    Imaging
    1. MRCP preferred over ERCP for diagnosis (noninvasive, less expensive, no risk of pancreatitis).
    Biopsy
    1. Liver biopsy not necessary to establish PSC diagnosis if cholangiographic findings are diagnostic.
    2. Liver biopsy recommended for suspected small duct PSC or to exclude other conditions (e.g. overlap with AIH).
    Labs
    1. AMA testing can help exclude PBC.
    2. Should test for elevated serum IgG4 at least once (PSC with elevated IgG4 may have rapidly progressive disease and/or potentially respond to steroids).

    Management

    ERCP
    1. ERCP + balloon dilation recommended if dominant stricture (CBD <1.5 mm, hepatic ducts <1.0 mm) and pruritus, and/or cholangitis.
    2. If dominant stricture on imaging, should do ERCP + cytology, biopsies, and FISH to exclude cholangiocarcinoma.
    3. Should give prophylactic antibiotics before ERCP to prevent post-ERCP cholangitis.
    4. Routine stenting after dilation of dominant stricture not required; short-term stenting may be required if severe stricture.
    General
    1. If mild pruritus, should treat with skin emollients and/or antihistamines.
    2. 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).
    3. Recommend screening for varices if advanced PSC or platelets < 150K × 10³/dL.
    4. At PSC diagnosis, recommend DEXA scan and repeat at 2-4-year intervals.
    5. If advanced PSC, should monitor for fat-soluble vitamin deficiencies (i.e., A, D, E, K).

    Special Situations

    Liver Transplant
    1. Liver transplant recommended (when possible) over medical therapy or surgical drainage in PSC with decompensated cirrhosis (prolongs survival).
    2. If MELD >14, should refer for liver transplant (5-year post-transplant survival 80-85%, but risk of PSC recurrence).
    Medical Treatment
    1. Should not use UDCA in doses >28 mg/kg/d (increased need for transplant, varices, and colonic neoplasia in PSC with UC).
    Special Situations
    1. If age <25 or higher-than-expected aminotransferases (5x ULN), recommend testing for AIH (~10% prevalence in PSC).
    2. If age <25 with AIH and serum ALP > 2x ULN, recommend MRCP to test for PSC.
    HPB Malignancies and Gallbladder Disease
    1. Consider US or MRCP + serial CA 19-9 every 6-12 months to screen for cholangiocarcinoma (10-year cumulative risk 8%).
    2. If GB polyps >8 mm, recommend cholecystectomy to prevent GB adenocarcinoma, and recommend annual US for GB cancer surveillance.
    PSC and IBD
    1. In PSC + IBD, recommend annual CRC screening with chromoendoscopy (4-5x increased dysplasia/cancer risk with PSC + IBD as compared to IBD without PSC).
    2. At time of PSC dx, full colonoscopy + bx recommended to evaluate for IBD.
    3. If no IBD on colonoscopy, some advocate repeat colonoscopy every 3–5 years.