Management of Primary Sclerosing Cholangitis and Extraintestinal Disorders in Patients with Ileal Pouches: A Systemic Review

Abstract

BACKGROUND: Restorative proctocolectomy with ileal pouch-anal anastomosis improves the quality of life in patients with ulcerative colitis by the removal of diseased large bowel and preservation of the natural route of defecation. Although the surgery may improve preexisting extraintestinal manifestations in the joint, skin, and eyes, extraintestinal manifestations, particularly primary sclerosing cholangitis can persist after colectomy. OBJECTIVES: A systemic review of diagnosis and treatment of liver, joint, skin, and eye manifestations in patients with restorative proctocolectomy and ileal pouch-anal anastomosis for ulcerative colitis DATA SOURCES: PubMed, Google Scholar, and Cochrane database. STUDY SELECTION: Relevant articles on primary sclerosing cholangitis and extraintestinal manifestations in ileal pouches published between Jan 2001 and July 2023 in English were included based on Preferred Reporting Items For Systemic Review and Meta-Analysis guidelines. INTERVENTION: Diagnosis and treatment of primary sclerosing cholangitis and extraintestinal manifestations in patients with restorative proctocolectomy and ileal pouch-anal anastomosis were included. MAIN OUTCOME MEASURES: Association between primary sclerosing cholangitis, extraintestinal manifestations, and inflammatory disorders of the pouch and their management. RESULTS: The presence of primary sclerosing cholangitis and extraintestinal manifestations is associated with pouchitis, particularly chronic pouchitis. Primary sclerosing cholangitis is associated with both chronic pouchitis and enteritis, and possible pouch neoplasia. However, disease severity and disease course of primary sclerosing cholangitis and pouchitis do not appear to parallel. Despite that oral vancomycin or budesonide have been used to treat primary sclerosing cholangitis-associated pouchitis, their impact on the disease course of primary sclerosing cholangitis is not known. Biological therapy for chronic inflammatory disorders of the pouch may be also beneficial for the concurrent extraintestinal manifestations of the joints, skin, and eyes. However, studies on the correlation between the severity of inflammatory pouch disorders and the severity of joint, skin, and eye diseases are lacking. LIMITATIONS: This is a qualitative, not quantitative review of case series and case reports. CONCLUSIONS: Primary sclerosing cholangitis and extraintestinal manifestations of the joints, skin, and eyes appear to be associated with inflammatory disorders of the ileal pouch. While the treatment of pouchitis does not seem to affect the disease course of primary sclerosing cholangitis, effective therapy of inflammatory pouch disorders, particularly with biologics, likely benefits concurrent disorders of the joints, skin, and eyes. See 20-minute symposium video.