Complications of Inflammatory Bowel Dx PBC and PBS

Question 1

pt with ulcerative colitis and cholestatic injury pattern on LFTs (high alkaline phosphatase and transaminases)

Answer 1

suggestive of primary sclerosing cholangitis

Question 2

Primary sclerosing cholangitis is associated with

Answer 2

ulcerative colitis (90% of with primary sclerosing cholangitis have underlying UC)

Question 3

What is primary sclerosing cholangitis?

Answer 3

chronic inflammation and fibrosis of the hepatic duct system causing strictures and cirrhosis. Progressive dx can cause pain, fatigue, pruritis, weight loss, and mild aminotransferase elevation <300. May have cholangitis.

Question 4

Diagnosis of Primary sclerosing cholangitis

Answer 4

cholangiography (see multifocal stricturing or focal dilation of hepatic bile ducts or “beads on a string” MRCP - MR cholangiopancreatography ERCP- Endoscopic retrograde cholangiopancreatography

Question 5

What is diagnostic on liver biopsy for primary sclerosing cholangitis?

Answer 5

see “onion skin” pattern of fibrous obliteration seen in minority of pts.

Question 6

When do we get biopsy of liver if concerned for primary sclerosing cholangitis?

Answer 6

rarely done but can be done in early disease or ppl who can’t get MRI

Question 7

Do we ever get a CT scan to look for cause of cholestasis?

Answer 7

helpful for malignancy or mass but rarely done to look for primary sclerosing cholangitis or malignancy and generally MRCP is more sensitive and specific. Also U/S preferred over CT scan for evaluating cholestatic liver injury and allows you to see anatomy and less radiation exposure.

Question 8

rectovaginal fistula and Crohn’s disease

Answer 8

20-30% of pts develop this and they present with a perianal fistula at time of diagnosis.

Question 9

treatment of asymptomatic fistula

Answer 9

no therapy as this will heal spontaneously.

Question 10

IBD related fistulas are treated

Answer 10

medically and only if this fails get surgical treatment

Question 11

pts with IBD related fistulas with mild symptoms are treated with

Answer 11

oral antibiotics with metronidazole and ciprofloxacin this often has a prolonged period of treatment of 6 to 12 months to risk relapse

Question 12

moderate to severe symptoms related to IBD related fistula

Answer 12

anti-TNF inhibitor, thiopurines or tacrolimus

Question 13

medication that has the best evidence for healing (both short term and long term) rectovaginal fistula

Answer 13

thiopurines and azathioprine/6 mercaptopurine try to avoid calcineurine inhibitors - cyclosporine or tacrolimus.

Question 14

algorithm for rectovaginal fistula

Answer 14

Question 15

what is primary biliary cholangitis?

Answer 15

autoimmune destruction of intrahepatic bile ducts and previously known as primary biliary cirrhosis. autoimmune destruction of intralobular bile ducts

Seen mostly middle aged women with insidious onset of fatigue and pruritis and progressive juandice and hepatosplenomegaly and cirrhosis

Question 16

primary biliary cholangitis or primary biliary cirrhosis clinical presentation

Answer 16

affects middle aged women, insidious onset of fatigue, pruritis, progressive jaundice and hepatomegaly and cirrhosis

see cutaneous xanthomas and xanthelasmas

Question 17

laboratory findings of primary biliary cholangitis

Answer 17

cholestatic pattern of liver injury (high alkaline phosphatase and aminotransferases)

see ANTI mitochrondrial antibody and severe hypercholesterolemia

Question 18

Treatment of primary biliary cholangitis

Answer 18

ursodeoxycholic acid (delays progression) and improves mortality. Don’t follow with antimitochondrial antibodies as this doesn’t correlate to dx response.

Follow LFTs every 3 -6 months while on ursodeoxycholic acid

needs liver transplant in advanced dx

Question 19

Complications of primary biliary cholangitis

Answer 19

malabsorption, fat soluble vitamin deficiencies, metabolic bone dx (osteoporosis and osteomalacia)

HCC

Question 20

severe hypercholesterolemia and abnormal skin pigmentation and severe fatigue and RUQ discomfort

Answer 20

consider primary biliary cholangitis or primary biliary cirrhosis because of pts may be asymptomatic at time of diagnosis.

need to check anti-mitochondrial antibodies

Question 21

positive anti-mitochondrial antibodies think:

Answer 21

primary biliary cholangitis or primary biliary cirrhosis

Question 22

ursodeoxycholic acid is used to treat

Answer 22

Primary biliary cholangitis or primary biliary cirrhosis

Follow LFTs every 3 -6 months while on this med.

Question 23

what is primary biliary cholangitis?

see chart

Answer 23

Question 24

what autoimmune diseases are associated with primary biliary cholangitis?

Answer 24

CREST syndrome, DM1 and RA

Question 25

what is seen on presentation with primary biliary cholangitis

Answer 25

see fatigue and generalized pruritis, see formation of xanthomas and xanthelasmas as they have marked HLD.

Get anti mitochrondial antibodies.

dont get confused with hematochromatosis because they will have elevated ALT/AST in hemachromatosis

Question 26

primary sclerosing cholangitis (PSC)

Answer 26

seen with IBD and UC. Has chronic progression of unknown etiology with inflammation, fibrosis and stricturing in the medium and large biliary ducts

Question 27

primary sclerosing cholangitis (PSC) presentation

Answer 27

fatigue, pruritus, and a cholestatic pattern of elevations in liver function tests. Continued bile duct destruction with PSC leads to end stage liver dx and portal HTN

Question 28

How to diagnose primary sclerosing cholangitis (PSC)/

Answer 28

ERCP/MRCP - cholangiogram will show multifocal narrowing and dilation of both intrahepatic and extrahepatic bile ducts. EUS won’t show anything. Liver biopsy is helpful for diagnosis and prognosis. Can reveal intraheaptic ductal obliteration with moderate lymphocytic infiltration and periductal onion skinning fibrosis

Question 29

primary sclerosing cholangitis (PSC) pts are at increased risk for:

Answer 29

colorectal cancer guidelines recommend get a screening colonoscopy with full biopsies at time of new diagnosis of PSC regardless if presence of other symptoms. Also increased risk for cholangiocarcinoma - annual monitoring for cancer antigen CA19-9 levels for screening.

Question 30

colonoscopy screening with IBD and PSC need

Answer 30

yearly colonoscopy

Question 31

pts with primary sclerosing cholangitis (PSC) alone and no IBD need colonoscopy screens every

Answer 31

5 years

Question 32

how to treat primary sclerosing cholangitis (PSC)

Answer 32

liver transplant. medical therapy doesn’t appear to slow the porgression of dx

Question 33

Primary biliary cholangitis PBC is not the same as primary sclerosing cholangitis (PSC). it presentation

Answer 33

is similar but primary biliary cholangitis affects women (90%) of time. Primary biliary cholangitis is autoimmune destruction of biliary ducts not FIBROSIS. biopsy shows monomuclear cell mediated destruction and periductal granulomatous inflammation of intrahepatic bile ducts.

Question 34

longterm complication of primary biliary cholangitis or PBC (previously primary biliary cirrhosis)

Answer 34

see metabolic bone dx or osteoporosis or osteomalacia

likely due to cholestasis leading to accmulaiton of substances that prevents bone turnover.

PBC can have significnat hypercholesterolemia (>1000 mg/dl) leads to xanthomas and xanthomas but thes pts are not at increased risk for CAD due to the elevation in HDL and mildly elevated LDL and VLDL.

Question 35

who does primary biliary cholangitis affect?

Answer 35

women ages 30-65

cholestatic injury pattenr on liver biomarkers and positive anti mitochrondrial antobides

50% can get osteoporosis or osteomalacia.

Question 36

after ileal resection from Crohns can see the development of

Answer 36

vitamin B12 deficiency

bile salt malabsorption - and subsequent malabsorption of fats and fat soluble vitamins

secretory diarrhea

Question 37

how to combat secretory diarrhea from ileal resection?

Answer 37

secretory diarrhea is from malabsorbed bile acids entering colon and causing colonic stimulation

give cholesytramine which lessens diarrhea by binding to bile acids.

Question 38

1st line treatment to mild to moderate UC is

Answer 38

5 aminosalicylate drugs like mesalamine.

Question 39

treatment of mild ulcerative proctitis confined to rectum who have initial remission on steroids can

Answer 39

do without maintenance therapy

but if have left sided colitis or more extensive dx, need to taper steroids and add a maintenance agent.

Question 40

algorithm for treatment of a rectovaginal fistula from Crohn’s dx

Answer 40

asymptomatic - observation

mild symptoms of rectovaginal fistula can be treated with prolonged period of antibiotics (ciprofloxacin and metronidazole) of 6-12 months with gradual tapering

moderate to severe symptoms need anti-TNF inhibitors, thiopruines and tacrolimus.