Complications of Inflammatory Bowel Dx PBC and PBS Flashcards
includes primary biliary sclerosis and primary biliary cholangitis and fistulas and etc.
pt with ulcerative colitis and cholestatic injury pattern on LFTs (high alkaline phosphatase and transaminases)
suggestive of primary sclerosing cholangitis
Primary sclerosing cholangitis is associated with
ulcerative colitis (90% of with primary sclerosing cholangitis have underlying UC)
What is primary sclerosing cholangitis?
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.
Diagnosis of Primary sclerosing cholangitis
cholangiography (see multifocal stricturing or focal dilation of hepatic bile ducts or “beads on a string” MRCP - MR cholangiopancreatography ERCP- Endoscopic retrograde cholangiopancreatography
What is diagnostic on liver biopsy for primary sclerosing cholangitis?
see “onion skin” pattern of fibrous obliteration seen in minority of pts.
When do we get biopsy of liver if concerned for primary sclerosing cholangitis?
rarely done but can be done in early disease or ppl who can’t get MRI
Do we ever get a CT scan to look for cause of cholestasis?
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.
rectovaginal fistula and Crohn’s disease
20-30% of pts develop this and they present with a perianal fistula at time of diagnosis.
treatment of asymptomatic fistula
no therapy as this will heal spontaneously.
IBD related fistulas are treated
medically and only if this fails get surgical treatment
pts with IBD related fistulas with mild symptoms are treated with
oral antibiotics with metronidazole and ciprofloxacin this often has a prolonged period of treatment of 6 to 12 months to risk relapse
moderate to severe symptoms related to IBD related fistula
anti-TNF inhibitor, thiopurines or tacrolimus
medication that has the best evidence for healing (both short term and long term) rectovaginal fistula
thiopurines and azathioprine/6 mercaptopurine try to avoid calcineurine inhibitors - cyclosporine or tacrolimus.
algorithm for rectovaginal fistula
what is primary biliary cholangitis?
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
primary biliary cholangitis or primary biliary cirrhosis clinical presentation
affects middle aged women, insidious onset of fatigue, pruritis, progressive jaundice and hepatomegaly and cirrhosis
see cutaneous xanthomas and xanthelasmas
laboratory findings of primary biliary cholangitis
cholestatic pattern of liver injury (high alkaline phosphatase and aminotransferases)
see ANTI mitochrondrial antibody and severe hypercholesterolemia
Treatment of primary biliary cholangitis
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
Complications of primary biliary cholangitis
malabsorption, fat soluble vitamin deficiencies, metabolic bone dx (osteoporosis and osteomalacia)
HCC
severe hypercholesterolemia and abnormal skin pigmentation and severe fatigue and RUQ discomfort
consider primary biliary cholangitis or primary biliary cirrhosis because of pts may be asymptomatic at time of diagnosis.
need to check anti-mitochondrial antibodies
positive anti-mitochondrial antibodies think:
primary biliary cholangitis or primary biliary cirrhosis
ursodeoxycholic acid is used to treat
Primary biliary cholangitis or primary biliary cirrhosis
Follow LFTs every 3 -6 months while on this med.
what is primary biliary cholangitis?
see chart
what autoimmune diseases are associated with primary biliary cholangitis?
CREST syndrome, DM1 and RA
what is seen on presentation with primary biliary cholangitis
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
primary sclerosing cholangitis (PSC)
seen with IBD and UC. Has chronic progression of unknown etiology with inflammation, fibrosis and stricturing in the medium and large biliary ducts
primary sclerosing cholangitis (PSC) presentation
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
How to diagnose primary sclerosing cholangitis (PSC)/
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
primary sclerosing cholangitis (PSC) pts are at increased risk for:
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.
colonoscopy screening with IBD and PSC need
yearly colonoscopy
pts with primary sclerosing cholangitis (PSC) alone and no IBD need colonoscopy screens every
5 years
how to treat primary sclerosing cholangitis (PSC)
liver transplant. medical therapy doesn’t appear to slow the porgression of dx
Primary biliary cholangitis PBC is not the same as primary sclerosing cholangitis (PSC). it presentation
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.
longterm complication of primary biliary cholangitis or PBC (previously primary biliary cirrhosis)
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.
who does primary biliary cholangitis affect?
women ages 30-65
cholestatic injury pattenr on liver biomarkers and positive anti mitochrondrial antobides
50% can get osteoporosis or osteomalacia.
after ileal resection from Crohns can see the development of
vitamin B12 deficiency
bile salt malabsorption - and subsequent malabsorption of fats and fat soluble vitamins
secretory diarrhea
how to combat secretory diarrhea from ileal resection?
secretory diarrhea is from malabsorbed bile acids entering colon and causing colonic stimulation
give cholesytramine which lessens diarrhea by binding to bile acids.
1st line treatment to mild to moderate UC is
5 aminosalicylate drugs like mesalamine.
treatment of mild ulcerative proctitis confined to rectum who have initial remission on steroids can
do without maintenance therapy
but if have left sided colitis or more extensive dx, need to taper steroids and add a maintenance agent.
algorithm for treatment of a rectovaginal fistula from Crohn’s dx
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.
