Clinical association between inflammatory bowel disease and primary sclerosing cholangitis: what changes after colectomy and liver transplantation?

Abstract

Inflammatory bowel disease (IBD), particularly ulcerative colitis (UC), is associated with several hepatobiliary manifestations, most importantly primary sclerosing cholangitis (PSC). The association between these entities is bidirectional, though not necessarily causal, making the underlying pathogenesis challenging to decipher. While not yet fully elucidated, current evidence suggests that genetic and immunological factors play key roles in the coexistence of IBD and PSC. In this review, we aim to provide a comprehensive analysis from a clinical perspective, evaluating the association between PSC, PSC-associated cholangiocarcinoma, orthotopic liver transplantation for PSC, IBD, colitis-associated neoplasia and restorative proctocolectomy with ileal pouch-anal anastomosis for UC. Despite efforts of data collection through population-based registries, much of the existing evidence is based on small cohorts, often with low event rates and limited follow-up durations. This makes it challenging to draw definitive conclusions. Acknowledging the variability and heterogeneity of prior studies, we aim to offer valuable insight for gastroenterologists and hepatologists managing this unique and often challenging scenario, which some authors consider a new entity: PSC-IBD. Longitudinal studies with extended follow-up periods are needed to better understand the disease course of PSC and UC, including the impact of medical therapy, the development, surveillance and management of neoplasia, and the outcomes of surgery for both bowel and liver diseases.

Keywords: Cholangiocarcinoma; Cholangitis, Sclerosing; Colectomy; Colitis, Ulcerative; Colorectal Neoplasms; Crohn Disease; Digestive System Surgical Procedures; Inflammatory Bowel Diseases; Liver Cirrhosis, Biliary; Liver Transplantation.

Figure 1. Association between primary sclerosing cholangitis and ulcerative colitis. (A) Severe ulcerative colitis with ulceration and spontaneous bleeding (the Mayo Endoscopy Score =3); (B) Colitis-associated high-grade dysplasia in a flat rectal lesion (green arrows); (C) Normal ileal pouch in restorative proctocolectomy with the tip of the ‘J’ (blue arrow) and pouch inlet (yellow arrow) highlighted; (D) Primary sclerosing cholangitis with dilated intrahepatic and extrahepatic bile ducts; (E) Primary sclerosing cholangitis-associated cholangiocarcinoma on PET scan; (F) Liver transplantation for primary sclerosing cholangitis now complicated by a duodenal B cell non-Hodgkin’s lymphoma (red arrow).

Figure 2. Concurrent primary sclerosing cholangitis and ulcerative colitis. (A) Backwash ileitis extending from diffuse extensive colitis with loss of vascularity, granular mucosa and exudates; (B) Widely patent (‘fish mouth’) ileocaecal valve (green arrow).

Figure 3. Ulcerative colitis-associated rectal adenocarcinoma in a patient with concurrent primary sclerosing cholangitis.

Figure 4. Inflammatory disorders of the ileal pouch in a patient with concurrent primary sclerosing cholangitis and ulcerative colitis. (A) Diffuse pouchitis with spontaneous bleeding, ulceration and mucosa nodularity; (B) Diffuse prepouch ileitis with ulceration and mucosal nodularity.

Figure 5. Liver transplantation on CT for primary sclerosing cholangitis in a patient with concurrent ulcerative colitis.

Figure 6. Left hepatectomy on CT for primary sclerosing cholangitis-associated cholangiocarcinoma in a patient with concurrent ulcerative colitis.

Figure 7. Recurrent primary sclerosing cholangitis with dilated intrahepatic and extrahepatic bile ducts on magnetic resonance cholangiopancreatography after liver transplantation in a patient with concurrent ulcerative colitis.