Published online Jul 28, 2020. doi: 10.3748/wjg.v26.i28.4126
Peer-review started: March 30, 2020
First decision: April 25, 2020
Revised: June 8, 2020
Accepted: July 15, 2020
Article in press: July 15, 2020
Published online: July 28, 2020
Primary sclerosing cholangitis (PSC) associated inflammatory bowel disease (IBD) is a unique form of IBD (PSC-IBD) with distinct clinical and histologic features from ulcerative colitis (UC) and Crohn disease (CD). In patients with PSC and IBD, the severity of the two disease processes may depend on each other.
To study the histologic and clinical features of PSC patients with and without IBD.
We assessed specimens from patients with UC (n = 28), CD (n = 10), PSC and UC (PSC-UC; n = 26); PSC and CD (PSC-CD; n = 6); and PSC and no IBD (PSC-no IBD; n = 4) between years 1999-2013. PSC-IBD patients were matched to IBD patients without PSC by age and colitis duration. Clinical data including age, gender, age at IBD and PSC diagnoses, IBD duration, treatment, follow-up, orthotopic liver transplantation (OLT) were noted.
PSC-UC patients had more isolated right-sided disease (P = 0.03), and less active inflammation in left colon, rectum (P = 0.03 and P = 0.0006), and overall (P = 0.0005) compared to UC. They required less steroids (P = 0.01) and fewer colectomies (P = 0.03) than UC patients. The PSC-CD patients had more ileitis and less rectal involvement compared to PSC-UC and CD. No PSC-CD patients required OLT compared to 38% of PSC-UC (P = 0.1). PSC-IBD (PSC-UC and PSC-CD) patients with OLT had severe disease in the left colon and rectum (P = 0.04).
PSC-UC represents a distinct form of IBD. The different disease phenotype in PSC-IBD patients with OLT may support liver-gut axis interaction, however warrants clinical attention and further research.
Core tip: This is a retrospective study evaluating biopsies and clinical features of primary sclerosing cholangitis (PSC) patients with and without inflammatory bowel disease (IBD) in comparison to subjects with ulcerative colitis (UC) or Crohn disease (CD). Patients with PSC-UC had a different disease distribution characterized by right sided colitis, a milder disease course with lower activity scores in biopsies, less need for colectomy, and less steroids compared to UC. PSC-CD patients were rare but had more ileal inflammation compared to PSC-UC. PSC-No IBD patients showed similar characteristics to PSC patients in general and only one patient received orthotopic liver transplantation (OLT) in this group. Ten PSC-UC patients received OLT in contrast to no patients with PSC-CD. The need for OLT in PSC-IBD (PSC-UC and PSC-CD) correlated with rectal involvement and higher activity scores in the left colon biopsies in comparison to patients without OLT. This may require clinical attention since the both the intestinal and liver disease seem to be “severe” in this group further supporting the importance of gut-liver interaction in these patients.