Endoscopic Management of Hepatobiliary Complications of Inflammatory Bowel Disease

List of Abbreviations

AASLD

The American Association of Study for Liver Disease

CC

Cholangiocarcinoma

CD

Crohn's disease

DC

Direct cholangioscopy

DIA

Digital image analysis

EASL

The European Association for the Study of the Liver

EIM

Extraintestinal manifestations

ERCP

Endoscopic retrograde cholangiopancreatography

EUS

Endoscopic ultrasound

FISH

Fluorescence in situ hybridization

FNA

Fine needle aspiration

IBD

Inflammatory bowel disease

IDUS

Intraductal ultrasound

IPAA

Ileal pouch-anal anastomosis

MRCP

Magnetic resonance cholangiopancreatography

pCLE

Probe-based confocal laser endomicroscopy

POC

Peroral cholangioscopy

PSC

Primary sclerosing cholangitis

PTC

Percutaneous transhepatic cholangiography

SOC

Single operator cholangioscopy

UC

Ulcerative colitis

Introduction

Inflammatory bowel disease (IBD) can be associated with a variety of extraintestinal manifestations (EIMs), which affect the eyes, skin, liver, and joints. Such EIMs are seen in about 25%–40% of patients with IBD and can occur in patients with both Crohn's disease and ulcerative colitis (UC). EIMs commonly involve liver, biliary tract, and pancreas ( Table 20.1 ), and primary sclerosing cholangitis (PSC) is the most important hepatopancreatobiliary manifestation of IBD. Of the various conditions listed in Table 20.1 , endoscopic intervention plays a particularly vital role in the diagnosis and treatment of PSC and cholangiocarcinoma (CC).

Table 20.1

List of Hepatopancreatobiliary Diseases in IBD

Biliary Tract/Gall Bladder Diseases

1.
Classical primary sclerosing cholangitis (PSC)

2.
Small duct type PSC

3.
PSC/autoimmune hepatitis overlap

4.
Cholangiocarcinoma

Hepatic Diseases

1.
Hepatic steatosis

2.
Drug-induced hepatitis

3.
Liver cirrhosis

4.
Granulomatous hepatitis

5.
Hepatic amyloidosis

6.
Liver abscess

7.
Primary biliary cirrhosis.

Pancreatic Diseases

1.
Gall stone pancreatitis

2.
Drug-induced pancreatitis

3.
Idiopathic acute and chronic pancreatitis

4.
Autoimmune pancreatitis.

Diagnosis of Primary Sclerosing Cholangitis

In the right setting, PSC can be reliably diagnosed with endoscopic imaging or CT or MRI imaging. Tissue diagnosis may be needed in small-duct PSC.

Definition

PSC is an autoimmune disease, which affects intrahepatic and extrahepatic biliary tree. It is characterized by progressive inflammation and fibrosis of biliary tract resulting in multifocal strictures and chronic cholestatic liver disease, which eventually progress to portal hypertension, cirrhosis, and hepatic decompensation.

Primary Sclerosing Cholangitis and Inflammatory Bowel Disease

PSC is very closely related to IBD, and about 60%–80% of patients with PSC have IBD. Among IBD, UC is more prevalent in PSC and is present in about 48%–86% patients of PSC. Conversely PSC is estimated to be present in about 2.4%–7.5% of patients with UC. UC patients with PSC demonstrate characteristic endoscopic and clinical features, as compared to UC without PSC. These include rectal sparring, pancolitis, back wash ileitis, mild clinical symptoms, higher risk of colorectal cancer, and increased risk of pouchitis after colectomy and ileal pouch-anal anastomosis (IPAA).

Diagnostic Criteria for Primary Sclerosing Cholangitis

Following are the essential diagnostic criteria of PSC: (1) cholestatic pattern of liver enzyme elevation (elevated alkaline phosphatase is the most common biochemical abnormality in PSC) ; (2) cholangiography (e.g., magnetic resonance cholangiopancreatography [MRCP], endoscopic retrograde cholangiopancreatography [ERCP], percutaneous transhepatic cholangiography [PTC]) showing multifocal strictures and segmental dilatations in bile ducts ( Fig. 20.1 ); (3) exclusion of secondary causes of sclerosing cholangitis ; and (4) in patients with cholestatic liver injury and characteristic findings on cholangiography, a liver biopsy is typically not required. Liver biopsy is reserved for patients with suspected small-duct PSC or an overlap syndrome with autoimmune hepatitis is suspected.

Figure 20.1, Cholangiogram showing a high-grade stricture measuring 3.5 cm in length involving the distal bile duct up to the cystic duct take off with moderate proximal biliary dilation. Severe intrahepatic primary sclerosing cholangitis changes are noted.

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