B0589 - Painless Jaundice With a Significantly Elevated CA 19-9: It Is Not Always Pancreaticobiliary Cancer

Monday, October 24, 2022

10:00 AM – 12:00 PM ET

Location: Crown Ballroom

Has Audio

Presenting Author(s)

David Jonason, MD

University of Minnesota Medical Center
Minneapolis, MN

David Jonason, MD¹, Aisha Mohamed, MS², Nicha Wongjarupong, MD³, Oyedele Adeyi, MD¹, Jeffrey H. Albrecht, MD¹, Guru Trikudanathan, MD¹
¹University of Minnesota Medical Center, Minneapolis, MN; ²University of Minnesota Medical School, Minneapolis, MN; ³University of Minnesota Medical Center, Hennepin County Medical Center, Minneapolis, MN

Introduction: Carbohydrate antigen 19-9 (CA 19-9) is often very high in pancreaticobiliary cancers but can be substantially elevated in benign diseases too. We present a case of painless jaundice with a significantly elevated CA 19-9 from vanishing bile duct syndrome (VBDS).

Case Description/Methods: 65-year-old white male with history of obesity, hypertension, hyperlipidemia, prior cholecystectomy and no family history of pancreatic cancer presented with two weeks of painless jaundice and 10 pounds unintentional weight loss. Physical exam noted scleral icterus and jaundice with a T.Bili of 23 mg/dL (Table 1). CT scan showed intrahepatic bile duct dilation and a 10 mm common bile duct without a discrete mass. CA 19-9 was elevated to 1,480 U/mL, concerning for malignancy. ERCP found a low-grade main bile duct stricture which was stented. Bile duct brushings and fine needle aspiration of the pancreas were negative for malignancy. Biliary dilation was resolved on follow-up MRCP but his bilirubin remained high. Infectious and autoimmune workup was unremarkable. Repeat cholangiography and brushings confirmed no obstruction or malignancy (Fig.1a). An infiltrative versus drug induced liver injury was suspected. EUS-guided liver biopsies revealed cholestasis with dilated bile canaliculi, hepatocytes with ballooning degeneration and a paucity of bile ductules. Cytokeratin-7 immunostaining demonstrated > 50% ductopenia, confirming VBDS (Fig.1b,c). Follow up at hepatology clinic determined triamterene-HCTZ, atorvastatin or omeprazole stopped during hospitalization to be the likely culprit. His CA 19-9, LFTs and symptoms improved over the following weeks (Table 1).

Discussion: VBDS is an acquired cholestatic liver disease that may mimic the presentation of pancreatic cancer. CA 19-9 can be substantially elevated through T-cell mediated destruction of small bile ducts causing impaired excretion, inflammatory production, and decreased clearance. Severity of liver damage depends on the duration of injury and degree of bile duct loss at the time of diagnosis. Prompt diagnosis is important to prevent cirrhotic progression, improve outcomes, and avoid unnecessary treatments. Recognizing its association with an elevated CA 19-9 may help with this, but is scarcely reported in the literature. CA 19-9 is not typically checked in cases of VBDS but is likely commonly elevated. Providers should be aware of this association when faced with a patient with painless jaundice and a significantly elevated CA 19-9 but no obvious malignancy.

Figure: Figure 1.
A) ERCP with cholangiogram showing a low-grade distal biliary stricture without significant upstream dilation. B) Liver biopsy with H&E stain, 500μm magnification showing small portal tracts with no bile ducts (red circle); note marked cholestasis (arrows) in dilated canaliculi and lack of ductular reaction. Also visible at this magnification is the ballooning degeneration of hepatocytes. C) Liver biopsy with cytokeratin 7 immunostain, 500μm magnification. Small portal tracts with no terminal/small bile ducts. Early signs of ductular reaction seen by hepatocytes taking up CK7 stain (arrows).

	Normal Range	Hospital Admission *	2 weeks	3 weeks **	4 weeks ***	7 weeks	13 weeks
Total bilirubin	0.2-1.3 mg/dL	23	22.7	22	19.7	4.7	1.3
Direct bilirubin	0.0-0.2 mg/dL	>13	n/a	14.9	14.6	4.2	0.6
AST	0-45 U/L	107	134	172	200	121	27
ALT	0-50 U/L	190	130	163	182	120	35
ALP	40-150 U/L	267	179	187	168	132	109
CA 19-9	0-35 U/mL	1480	n/a	n/a	85	49	n/a

Table: LFT Trends During the Course of Presentation.

AST, aspartate aminotransferase; ALT, alanine aminotransferase; ALP, alkaline phosphatase; CA19-9, carbohydrate antigen 19-9; n/a, not available.
* ANA, anti-AMA, anti-SMA, anti-mitochondrial, anti-TTG, anti-LKM 1/2 IgG, and EBV IgG antibodies were negative. Serum IgA, A1AT, ceruloplasmin, TSH, iron levels, hepatitis panel, serum protein electrophoresis and acetaminophen levels were normal. Acetaminophen, multivitamin, iron, triamterene- HCTZ, omeprazole and atorvastatin were stopped on discharge.
** Three weeks post hospitalization. Repeat EUS/ERCP showed biliary decompression. VBDS diagnosed on liver biopsies.
*** Seen in liver clinic. Started ursodiol 2000mg daily.

Disclosures:

David Jonason indicated no relevant financial relationships.

Aisha Mohamed indicated no relevant financial relationships.

Nicha Wongjarupong indicated no relevant financial relationships.

Oyedele Adeyi indicated no relevant financial relationships.

Jeffrey Albrecht indicated no relevant financial relationships.

Guru Trikudanathan: Boston Scientific Corporation – Consultant.

David Jonason, MD¹, Aisha Mohamed, MS², Nicha Wongjarupong, MD³, Oyedele Adeyi, MD¹, Jeffrey H. Albrecht, MD¹, Guru Trikudanathan, MD¹. B0589 - Painless Jaundice With a Significantly Elevated CA 19-9: It Is Not Always Pancreaticobiliary Cancer, ACG 2022 Annual Scientific Meeting Abstracts. Charlotte, NC: American College of Gastroenterology.