Pericholangitis Treatment & Management

Updated: Jun 22, 2016
  • Author: Robert P Myers, MD, FRCPC; Chief Editor: BS Anand, MD  more...
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Treatment

Approach Considerations

No effective medical treatment exists for pericholangitis; liver transplantation is the only effective treatment. No effective preventive therapy exists for pericholangitis. Neither colectomy in patients with ulcerative colitis (UC) nor medical management of associated inflammatory bowel disease (IBD) prevents the development of pericholangitis. [13, 14, 15]

Patients with complications of end-stage pericholangitis, such as ascites, spontaneous bacterial peritonitis, portal hypertensive bleeding, and hepatic encephalopathy, should be treated like patients with other causes of chronic liver disease. Transfer patients with end-stage pericholangitis to a center specializing in liver transplantation. Failure to do so is a potential medicolegal pitfall.

A gastroenterologist should be consulted for the management of associated IBD, including screening for colorectal dysplasia and cancer. An endocrinologist and a metabolism specialist should be consulted for the management of associated osteopenia or osteoporosis.

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Pharmacologic and Supplemental Therapy

Several medications, including penicillamine, colchicine, corticosteroids, azathioprine, methotrexate, nicotine, and pentoxifylline, have been tried for primary sclerosing cholangitis (PSC) and have proved ineffective, though they have not been specifically tested in patients with pericholangitis. [16, 17, 18] Ursodeoxycholic acid may have a role in this disorder.

For patients with documented deficiencies of fat-soluble vitamins (ie, A, D, E, or K), initiate vitamin replacement. For patients who are osteopenic, prescribe calcium (>1000 mg/day) and vitamin D (800 IU/day).

Patients who are osteoporotic should receive bisphosphonate therapy. These agents can cause esophageal irritation when taken orally, which raises concerns about their safety in patients with esophageal varices. Patients with documented esophageal varices probably should receive intermittent intravenous (IV) infusions of bisphosphonates such as pamidronate.

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Liver Transplantation

Liver transplantation is the only proven effective treatment for PSC. Preliminary evidence suggests that patients with late pericholangitis also do well with liver transplantation. Most series have reported a dismal prognosis for liver transplantation if cholangiocarcinoma is present, and these results discourage transplantation in this situation.

Compared with patients who undergo liver transplantation for other indications, patients with PSC seem to have a higher incidence of chronic ductopenic rejection after transplant surgery. Large-duct PSC recurs in approximately 20% of liver allografts. Recurrence of pericholangitis has yet to be reported.

Those with long-standing IBD should undergo surveillance colonoscopy every 1-2 years. Patients with IBD who have received a liver transplant for pericholangitis may be at increased risk as a consequence of the effects of immunosuppression. They require more intense colonoscopic surveillance.

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