Northeast Florida Medicine Journal, Winter 2015

Vol. 66, No. 4 2015

Northeast Florida Medicine

DCMS online

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Inflammatory Bowel Disease

PSC can precede the initial diagnosis of IBD. In addi-

tion, some patients are diagnosed with PSC several years

after having proctocolectomy. Therefore, patients with

PSC should always undergo a colonoscopy to evaluate

for inflammatory bowel disease. Typically what is seen is

involvement of the colon with rectal sparing. At times,

there may be some ileitis as well. These patients present

with fatigue and abdominal pain, weight loss, pruritus,

and then intermittent bouts of jaundice. Abnormal liver

function tests include markedly elevated alkaline phos-

phatase and gamma-glutamyl transpeptidase (GGTP).

Bilirubin levels can slowly rise from 2 to 20. There may

be a number of autoantibodies detected including ANA,

anti-smooth muscle antibodies, and anti p-ANCA in

close to 80 percent of patients.

MRCP or endoscopic

retrograde cholangiopancreatography (ERCP) can be

diagnostic.

Typically ERCP is reserved for patients that

are thought to have a dominant stricture that can be

investigated, brushed for malignant cytology, dilated, or

even transiently stented for decompressive relief. (Figure

5) Patients with small duct PSC may need a liver biopsy

to be confirmatory. Total colectomy does not seem to

make a difference as to the clinical course of PSC. For

patients with significant cholestasis, if an ultrasound is

normal, and there do not appear to be any major drugs

to incriminate, and if serological tests for other primary

liver diseases are negative, then the probability of PSC

increases. If MRCP is normal and PSC is still suspected, a

liver biopsy may be very appropriate and safe to perform,

rather than ERCP with its remote risk of pancreatitis.

PSC substantially increases the risk of both cholangiocar-

cinoma and colorectal carcinoma. Annual colonoscopy is

recommended once the diagnosis of PSC is confirmed. The

severity of UC is not related to the severity of the PSC.

Figure 5:

PSC in chronic UC – Endoscopic retrograde

cholangiopancreatography (ERCP) Image

PSC appears to respond to ursodeoxycholic acid, which

improves abnormal liver function tests in cholestasis, but

does not affect the overall course of the disease. A dose of

20 mg/kg may improve prognosis. It is possible that it also

reduces the risk of colonic cancer in these patients. However,

there is no firmevidence that ursodiol has a convincing effect

on the course of this disease.

In addition, a study on the

long-term use of high dose ursodeoxycholic acid revealed an

increased risk of colonic neoplasia in patients with UC and

PSC.

Therefore, the use of ursodiol for the management of

PSC is currently not recommended in general.

Dilation of dominant strictures at the time of ERCP

may improve cholestatic symptoms. On rare occasion, the

placement of stents, preferably fully covered expandable

metal stents, may be of benefit. Most of all, the goal is to

avoid colonization of the biliary tree from duodenal con-

tents as much as possible. Orthotopic liver transplantation

is the therapy of choice for patients with end stage PSC

and carries a fairly favorable five year survival rate of 80

percent.

However, the risk of developing cholangiocar-

cinoma in these patients is paramount and is on the mind

of every endoscopist involved with this disorder.

Autoimmune hepatitis, with or without overlap with

PSC, is more common in patients with UC than CD.

granulomatous hepatitis is a rare manifestation of patients

with Crohn’s. Cholelithiasis occurs in up to 30 percent of

patients with IBD, especially in those with ileal Crohn’s or

after an ileal resection.

This is explained by the increased

enterohepatic circulation of bilirubin and augmented re-

absorption of bilirubin, caused by increased colonic bile

salts in these patients. The increased risk of developing

cholesterol gallstones might be caused by abnormal bile salt

absorption and cholesterol supersaturated bile. In addition,

reduced gallbladder motility in patients with IBDwho have

periods of fasting or may require total parenteral nutrition

also seems to promote the development of cholelithiasis

in these sick patients. Nonalcoholic fatty liver disease and

nonalcoholic steatohepatitis (NASH) are often diagnosed

in patients with IBD at a prevalence of close to 10 percent

of patients with UC and up to 20 percent in those with

Crohn’s.

Corticosteroids, methotrexate, Imuran and

Total Parenteral Nutrition (TPN) may all promote the

development of fatty liver disease in these patients. Patients

with IBD have an increased risk of developing a severe

complication of non-Hodgkin’s lymphoma. Several cases

of hepatosplenic T cell lymphoma have been reported in

patients with IBD, mostly in those who have been treated

with a combination of anti-TNF therapy, the thiopurines,

and corticosteroids. In view of the fatal course of this

complication, the long-term use of a combination of these

drugs should be used with extreme caution. Finally, PBC,

chronic hepatitis, and portal vein thrombosis may be seen

with greater prevalence in patients with IBD.

Pancreatic manifestations in IBD can include acute pan-

creatitis which may be precipitated by some of the drugs