Abstract
Objectives The clinical/colonoscopic features of ulcerative colitis (UC) associated with primary sclerosing cholangitis (PSC), the prognostic impact of UC, and the utility of UC screening in PSC patients are unknown. We characterized UC associated with PSC and assessed UC's impact on the prognosis of PSC and the importance of colonoscopic UC screening in PSC patients. Methods We retrospectively analyzed the cases of 77 patients treated for PSC at a single center (April 2000–July 2019). We reviewed the clinical/colonoscopic profiles of the concurrent UC patients and compared the clinical profiles, survival, and primary causes of death between the patients with/without UC (n = 35/n = 42). The details of all patients' colonoscopies were reviewed. Results The concurrent UC group: 17 men, 18 women, diagnosed with PSC at the mean (SD) age of 36 (17) years; 21 patients (60%) had no UC symptoms. Colonoscopy revealed pancolitis in all patients, predominantly affecting the right-sided colon in 30 patients (86%). Lesions were scattered. Backwash ileitis (n = 13, 37%) and rectal sparing (n = 18, 51%) were observed. Most patients had mild UC; some had moderate or more severe UC (median Ulcerative Colitis Endoscopic Index of Severity (UCEIS) score 2; range, 1–5). Ludwig's stage determined by liver biopsy did not correlate with the Mayo endoscopic score for UC. The patients with UC were diagnosed with PSC at a significantly younger age than those without UC (mean (SD), 36 [17] years vs. 55 [19] years, p < 0.0001) and had a significantly higher 5-year survival rate (97.1% vs. 70.5%, p = 0.0028). UC was detected in 19 of 34 asymptomatic patients (56%) who underwent colonoscopy screening. Conclusions Our cohort's clinical/colonoscopic features of UC associated with PSC are more moderate or severe UC than previous cases. The coexistence of UC might affect the prognosis of PSC. In this regard, colonoscopy in PSC patients is an important examination for determining prognosis. There is also asymptomatic UC in patients with PSC. In this regard, screening for colonoscopy in PSC patients is essential. When a diagnosis of PSC is made, immediate colonoscopy is a priority with UC complications in mind.
Highlights
Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease characterized by diffuse multiple structuring of the intrahepatic and extrahepatic bile ducts [1]
To more clearly determine the importance of diagnosing and treating inflammatory bowel disease (IBD) associated with PSC, we studied the clinical and colonoscopic features of ulcerative colitis (UC) associated with PSC, the impact of UC on the prognosis of underlying PSC, and the clinical significance of colonoscopic UC screening in PSC patients
We collected data on the following parameters: sex, age at the diagnosis of PSC, age at the diagnosis of UC, the diagnostic sequence of UC in relation to PSC, the presence/absence of symptoms of UC, the extent of UC, the location of inflammation, the presence/absence of backwash ileitis [16], the presence/absence of rectal sparing [17], the Lichtiger Clinical Activity Index (CAI) [18], the endoscopic UC severity score, Ludwig’s stage determined by liver biopsy [21], and the treatments provided for UC and PSC
Summary
Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease characterized by diffuse multiple structuring of the intrahepatic and extrahepatic bile ducts [1]. PSC has a poor prognosis because of the high risk of recurrence after liver transplantation [4], which is the only radical treatment for this condition. There are no effective medical treatments for PSC. PSC is closely related to inflammatory bowel disease (IBD) [1,2,3,4,5]. The most common type of IBD associated with PSC is ulcerative colitis (UC) [6,7,8]. Because UC is becoming more prevalent in Japan [9], IBD associated with PSC may become more prevalent in Japan. It is likely that it will become important to diagnose and treat IBD in PSC patients in Japan. UC associated with PSC is mostly mild and is occasionally followed up as nonspecific colitis. Clinicians should be aware of these and other differences between UC with and without underlying PSC
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