Primary Sclerosing Cholangitis With Ulcerative Colitis Presents as a Unique Disease Phenotype

Primary sclerosing cholangitis (PSC). This chronic cholestatic liver disease is characterised by an inflammation and fibrosis of the biliary tracts.
Researchers studied the histologic and clinical features of primary sclerosing cholangitis patients with and without IBD to predict disease severity.

Patients with primary sclerosing cholangitis (PSC) and ulcerative colitis (UC) had distinct disease symptoms compared with patients with PSC or UC alone. These findings, from a retrospective study, were published in the World Journal of Gastroenterology.

Patients with UC (n=28), Crohn disease (CD; n=10), PSC without inflammatory bowel disease (IBD) (n=4), PSC-UC (n=26), and PSC-CD (n=6) had colonic biopsy and resection specimens collected between 1999 and 2013 at the Barnes-Jewish Hospital in the United States. Patients with and without PSC were matched for age and CD or UC disease duration.

Among patients with UC, the PSC-UC and UC groups did not differ significantly for sex, diagnosis age, or dysplasia rates. Fewer patients with PSC-UC were treated with steroids (31% vs 68%; P =.01) or had colectomies (38% vs 68%; P =.03) compared with patients with UC alone.

The biopsy data indicated that in patients with PSC-UC, disease was more likely to be confined to the right side of the colon (29% vs 4%; P =.03), activity scores were lower in the rectum (P =.03 vs P =.0006), there was less basal plasmacytosis in the left colon and rectum (P =.01 vs P =.0004), and overall inflammation was lower across all sites (P =.0005), compared with UC alone, respectively.

Among patients with CD, the PSC-CD and CD groups were well balanced for age at diagnosis, CD disease duration, colectomy rates, and dysplasia rates. The PSC-CD group had more men (P =.03), and fewer were treated with anti-tumor necrosis factor (TNF) therapy (17% vs 78%; P =.04) compared with patients with CD alone. Comparison of biopsy specimens between patient groups did not reveal any significant differences.

Patients with PSC-UC and PSC-CD had similar biopsy results, except that patients with PSC-CD had higher disease activity scores in the terminal ileum (1.0 vs 0.31, respectively; P =.01) compared with patients with PSC-UC. Patients with PSC and no IBD had normal colonic biopsy specimens.

Patients with PSC-IBD who had an orthotopic liver transplantation (OLT) presented with more rectal involvement (P =.04), a more inflamed rectum (P =.04), and a more inflamed left colon (P =.04) compared with patients with PSC-IBD not requiring OLT.

This study was limited by its small sample size; however, due to the rarity of these diseases, collection of such specimens was inherently difficult.

The study authors concluded that patients with PSC-UC had a distinct disease phenotype compared with patients with either disease alone. Furthermore, patients with PSC-IBD who had a liver transplant presented with more severe IBD symptoms than patients with the same disease state not requiring a transplant.

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Reference

Aranake-Chrisinger J, Dassopoulos T, Yan Y, et al. Primary sclerosing cholangitis associated colitis: characterization of clinical, histologic features, and their associations with liver transplantation. World J Gastroenterol. 2020;26(28):4126-4139. doi: 10.3748/wjg.v26.i28.4126.