October 31 2013 Fellow David Tang MD Faculty Clark Hair MD Case Presentation 30 year old Hispanic woman 2010 Diagnosed with Ulcerative Colitis Started on Mesalamine and Azathioprine ID: 678624
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Slide1
Gastroenterology Grand Rounds
October 31, 2013Fellow: David Tang, M.D.Faculty: Clark Hair, M.D.Slide2
Case Presentation
30 year old Hispanic woman[2010] Diagnosed with Ulcerative ColitisStarted on Mesalamine
and Azathioprine
Three flares since diagnosis each resolved
after starting steroid taperSlide3
[8/26] Presented to
Houston area hospital with RUQ abdominal pain x 4 days
AST
144
ALT
266
Alk Phos522Total Bilirubin4.1Direct Bilirubin3.6Total Protein7.2Albumin3.7
Case PresentationSlide4
[8/26] RUQ Abdominal UltrasoundHydropic
gallbladder with an associated stone at the gallbladder neck. No intrahepatic or extrahepatic biliary ductal dilation.[8/29] MRCP
Subtle contour irregularity within the intrahepatic biliary system suggesting PSC
1.2 cm segment of proximal
extrahepatic
common bile duct is smoothly narrowed with possible adjacent are of soft tissue prominence
Case PresentationSlide5
[8/30] ERCPSlide6
[9/4] ColonoscopySlide7
[9/23] EUSSlide8
Courtesy of Dr.
Zarrin-Khameh
, PathologySlide9
Courtesy of Dr.
Zarrin-Khameh
, PathologySlide10
Cholangiocarcinoma
Pathological DiagnosisSlide11
Clinical Questions
What is the epidemiological relationship of Cholangiocarcinoma to PSC?
Is concomitant IBD a risk for developing
Cholangiocarcinoma
? What are the other risk factors?
What is the utility of CA 19-9 in the diagnosis of
Cholangiocarcinoma? How should it be used in screening patients with PSC?Slide12
Prevalence of CCA in PSC
Cohort Size
Prevalence
Median
Follow Up
Broome
3058%5.3 yearsBergquist 60413.3%5.7 yearsBoberg
394
12.2%
4.7
years
Burak
161
6.8%
11.5 years
Tischendorf
273
14.3%
6.3 years
Fevery
123
6.9%
9 years
Charatcharoenwitthaya
230
10%
4.1 years
Claessen
211
7.1%
9 yearsSlide13
Prevalence of IBD
in PSC
Size
Population
Concomitant
IBD
Concomitant UCConcomitant CDBerquist604Sweden
79%
69%
7%
Boberg
394
Europe
82%
65%
-
Weisner
174
Minnesota
71%
-
-
Takikawa
192
Japan
21%
19.8%
1%
Kochlar
18
India
-
50%
-Slide14
Does concomitant IBD increase risk of CCA in PSC?
Multicenter European retrospective cohort study with 394 PSC patientsMedian follow up 4.7 years12.2% of patients developed
CholangiocarcinomaSlide15
Does concomitant IBD increase risk of CCA in PSC?Slide16
Predisposing factors for
Cholangiocarcinoma
in PSC
Retrospective cohort study of 161 patients with PSC at Mayo Clinic
Median follow up 11.5 years
6.8 % of patients developed
CholangiocarcinomaHistory of variceal bleeding, lack of symptoms at baseline, and proctocolectomy associated with increased risk of CholangiocarcinomaSlide17
Predisposing factors for Cholangiocarcinoma
in PSC
Multicenter case-control study of 26 cases of
Cholangiocarcinoma
in PSC matched with 87 patients with PSC alone
Duration of IBD was not a significantly associated with
Cholangiocarcinoma from 8 cases and 33 controlsSlide18
Predisposing factors for
Cholangiocarcinoma
in PSCSlide19
Predisposing factors for
Cholangiocarcinoma
in PSCSlide20
Utility of CA 19-9 in Diagnosis of Cholangiocarcinoma
AUC = 0.84
CA 19-9 cut off of > 100 U/mL was 75% sensitive and 80% specific for
CholangiocarcinomaSlide21
AUC = 0.76
Utility of CA 19-9 in Diagnosis of
Cholangiocarcinoma
Cross sectional study of 333 patients with PSC from 1984 to 1997
13% with
Cholangiocarcinoma
CA 19-9 cut off of > 180 U/mL was 75% sensitive and 97% specific for CholangiocarcinomaSlide22
AUC = 0.95
Utility of CA 19-9 in Diagnosis of
Cholangiocarcinoma
Retrospective cohort study of 208 patients at Mayo clinic who had PSC and serial CA 19-9 measurements
Mean follow up 2.6 years
CA 19-9 cut off of > 129 U/mL was 78.6% sensitive and 98% specific for
CholangiocarcinomaOnly 2 out of 14 patients with Cholangiocarcinoma were resectableSlide23
AUC
0.79
Cutoff
20 U/mL
Sn
78
Sp6723/2304.1 yearsUtility of CA 19-9 in Diagnosis of CholangiocarcinomaSlide24
Combination of cross sectional imaging and CA 19-9 for diagnosis of
CholangiocarcinomaSlide25
Combination of cross sectional imaging and CA 19-9 for diagnosis of CholangiocarcinomaSlide26
Proposed algorithm for screening and diagnosis of
Cholangiocarcinoma