Primary sclerosing cholangitis: Inflammatory bowel disease and colorectal cancer
- Kris V Kowdley, MD, FACP, FACG, FASGE, AGAF
Kris V Kowdley, MD, FACP, FACG, FASGE, AGAF
- Director, Liver Care Network and Organ Care Research
- Swedish Medical Center
- Seattle, WA
Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease. The majority of patients with PSC have underlying inflammatory bowel disease (IBD). Patients with concurrent PSC and IBD have an increased risk of colorectal cancer (CRC) [1-6]. This topic review will focus on the epidemiology, pathogenesis, clinical features, diagnosis, and management of IBD in patients with PSC in addition to the risk of CRC and guidelines for CRC surveillance. CRC surveillance in patients with IBD is presented separately. (See "Colorectal cancer surveillance in inflammatory bowel disease".)
INFLAMMATORY BOWEL DISEASE
Epidemiology — The prevalence of inflammatory bowel disease (IBD) in patients with primary sclerosing cholangitis (PSC) approaches 90 percent [7,8]. Ulcerative colitis (UC), Crohn disease (CD), and indeterminate colitis of the colon account for 80, 10, and 10 percent, respectively . (See "Primary sclerosing cholangitis: Epidemiology and pathogenesis", section on 'PSC and inflammatory bowel disease'.)
Clinical features — Colitis in patients with PSC often presents at an earlier age as compared with patients with IBD alone . The colitis usually has a mild or quiescent course. Patients are often asymptomatic but in rare cases present with rectal bleeding. (See "Clinical manifestations, diagnosis, and prognosis of ulcerative colitis in adults", section on 'Colitis' and "Clinical manifestations, diagnosis and prognosis of Crohn disease in adults", section on 'Clinical manifestations'.)
Following colectomy, patients can present with stomal and peristomal variceal bleeding secondary to portal hypertension associated with PSC. Patients with proctocolectomy with ileal pouch anal anastomosis also have an increased risk of pouchitis [9,11,12]. Symptoms of pouchitis include increased stool frequency, urgency, abdominal cramps, pelvic pressure, tenesmus, and night-time fecal seepage to incontinence. (See "Routine care of patients with an ileostomy or colostomy and management of ostomy complications", section on 'Stomal bleeding' and "Pouchitis: Epidemiology, clinical manifestations, and diagnosis", section on 'Clinical manifestations'.)
The course of colitis following liver transplantation for PSC is variable. Although the colitis may remain quiescent in some patients, other patients may have severe symptoms (10 or more stools per day with severe cramps and continuous bleeding) despite the immunosuppression used for the transplant [13-15]. (See "Primary sclerosing cholangitis in adults: Management", section on 'Inflammatory bowel disease' and "Clinical manifestations, diagnosis, and prognosis of ulcerative colitis in adults", section on 'Colitis'.)
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- INFLAMMATORY BOWEL DISEASE
- Clinical manifestations
- - Clinical features
- - Endoscopic features
- Differential diagnosis
- COLORECTAL CANCER
- Clinical features
- - Colorectal cancer screening
- PSC and IBD
- PSC without IBD
- - Chemoprevention
- SUMMARY AND RECOMMENDATIONS