Liver & intrahepatic bile ducts
Biliary tract disease
Recurrent pyogenic cholangitis
Author: Raul S. Gonzalez, M.D.
Editor-in-Chief: Debra L. Zynger, M.D.
Last author update: 10 December 2020
Last staff update: 5 August 2022
Copyright: 2002-2024, PathologyOutlines.com, Inc.
PubMed Search: Recurrent pyogenic cholangitis
Table of Contents
Definition / general | Essential features | Terminology | ICD coding | Epidemiology | Etiology | Clinical features | Radiology description | Radiology images | Prognostic factors | Case reports | Treatment | Clinical images | Gross images | Microscopic (histologic) description | Microscopic (histologic) images | Sample pathology report | Differential diagnosis | Board review style question #1 | Board review style answer #1 | Board review style question #2 | Board review style answer #2Cite this page: Gonzalez RS. Recurrent pyogenic cholangitis. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/liverorientalcholangiohepatitis.html. Accessed April 23rd, 2024.
Definition / general
- Recurrent attacks of obstructive cholangitis due to Escherichia coli infection in the setting of liver fluke infection or hepatolithiasis
Essential features
- Recurrent bile duct injury due to flukes, hepatoliths or bacterial infection
- Treatment often requires surgery
- Patients are at increased risk for cholangiocarcinoma
Terminology
- Sometimes termed “Oriental cholangiohepatitis” or “primary hepatolithiasis” (Adv Anat Pathol 2011;18:318)
ICD coding
- ICD-10: K83.09 - other cholangitis
Epidemiology
- Generally occurs in Southeast Asia or in patients from that region but may occur elsewhere (Neth J Med 2016;74:401, Hawaii Med J 2007;66:9, World J Surg 2005;29:858)
Etiology
- Patients often have liver flukes (e.g. Clonorchis sinensis) or hepatolithiasis as the initial inciting factor (J Emerg Med 2012;43:e107)
- E. coli, found in the bile of most patients during acute attacks, appears to be the second inciting factor
Clinical features
- Usually occurs in young adults of either sex; more common in rural areas (Abdom Radiol (NY) 2017;42:46)
- Symptoms: acute or recurrent bouts of suppurative cholangitis accompanied by abdominal pain, fever and jaundice (Semin Liver Dis 2011;31:33)
- Patients are at increased risk for developing cholangiocarcinoma
Radiology description
- Dilation of extrahepatic bile ducts with relatively mild / no dilation of intrahepatic ducts, localized dilatation of the lobar or segmental bile ducts, increased periportal echogenicity, segmental hepatic atrophy, gallstones (Clin Res Hepatol Gastroenterol 2012;36:515, Abdom Radiol (NY) 2017;42:46)
- Peripheral ducts may taper abruptly (Abdom Radiol (NY) 2017;42:2964)
- Localized intrahepatic segmental ductal stenosis may be present, especially in the lateral segment of the left lobe or posterior segment of the right hepatic lobe
Radiology images
Images hosted on other servers:
Cholangiogram
with stones
CT with stones
Prognostic factors
- Patients with bilateral disease or who do not undergo operative treatment fare worse (ISRN Surg 2013;2013:536081)
Case reports
- 39 year old Korean woman with acute cholecystitis (Case Rep Gastrointest Med 2017;2017:8575674)
- 48 year old Vietnamese woman with abdominal pain (Dig Dis Sci 2016;61:3147)
- 74 year old South Asian man with symptoms concerning for cholangiocarcinoma (Int J Surg Case Rep 2012;3:235)
Treatment
- Some cases require surgery (Int J Surg. 2014;12:789, HPB (Oxford) 2016;18:821)
- Other cases may be managed endoscopically (stone extraction, sphincterotomy, stent placement) (J Community Hosp Intern Med Perspect 2015;5:27858, Dig Dis Sci 1997;42:865)
Clinical images
Images hosted on other servers:
Intrahepatic stone
Gross images
Images hosted on other servers:
Duct dilatation and stones
Microscopic (histologic) description
- Dilation and fibrosis of bile ducts with acute or chronic inflammation (Adv Anat Pathol 2011;18:318)
- Other findings include hepatic / intraductal abscesses and secondary sclerosing cholangitis
- Liver flukes or bile duct stones may be seen
Microscopic (histologic) images
Contributed by Raul S. Gonzalez, M.D.
Hepatolithiasis
Periductal fibrosis
Duct inflammation
Sample pathology report
- Liver, right lobe, partial hepatectomy:
- Bile ducts with acute and chronic inflammation, fibrosis and hepatolithiasis (see comment)
- Background liver parenchyma with reactive change
- Comment: The findings are compatible with the patient’s reported history of recurrent pyogenic cholangitis. The examined sections are negative for dysplasia or malignancy.
Differential diagnosis
- Caroli syndrome:
- Congenital and therefore usually presents earlier in life
- Primary sclerosing cholangitis:
- Lesser degree of inflammation
- Hepatoliths generally not present
- Segmental cholangiectasia:
- High clinical and pathologic overlap, except this disease occurs in non-Asian patients (Hum Pathol 2015;46:426)
Board review style question #1
- A young Asian patient presents with recurrent abdominal pain. Workup indicates hepatolithiasis with obstructive cholangitis. Which bacteria is most likely contributing to his symptoms?
- Escherichia coli
- Mycobacterium tuberculosis
- Pseudomonas aeruginosa
- Shigella sonnei
- Staphylococcus aureus
Board review style answer #1
Board review style question #2
- A 50 year old Asian woman is found to have a liver mass. Biopsy is consistent with cholangiocarcinoma. She endorses a history of recurrent abdominal pain and fevers. What is the most likely underlying diagnoses?
- Benign recurrent intrahepatic cholestasis
- Crohn’s disease
- Longstanding drug induced liver injury
- Primary biliary cholangitis
- Recurrent pyogenic cholangitis
Board review style answer #2
