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DDx

Diagnostic Considerations

The differential diagnosis of primary sclerosing cholangitis (PSC) includes congenital diseases (eg, Caroli disease, choledochal cysts) and secondary cholangiopathy, as observed in patients with collagen vascular diseases (eg, systemic lupus erythematosus, rheumatoid arthritis, systemic sclerosis) and in those with infiltrative diseases (eg, mediastinal fibrosis, Riedel thyroiditis, eosinophilic cholangitis, histiocytosis X). Infectious causes from parasitic, fungal, viral, or bacterial infections or from recurrent cholangitis itself, especially in patients who are immunocompromised, can cause multifocal liver abscesses that lead to a PSC-like appearance of the bile duct.

Biliary S100A9 levels may be a useful marker for PSC activity, and its implication in inflammation and carcinogenesis warrants further investigation.^[28]

The most important differential diagnosis is from cholangiocarcinoma. CA 19-9 levels above 130 U/mL may suggest the diagnosis of cholangiocarcinoma, which can be established by brush cytology (endoscopic or percutaneous transhepatic).

Differential Diagnoses

Abdominal Vascular Injuries
Acalculous Cholecystitis
Autoimmune Hepatitis
Bile Duct Strictures
Bile Duct Tumors
Biliary Obstruction
Biliary Trauma
Cholangiocarcinoma
Cholangitis
Common Bile Duct Stones
Gallbladder Cancer
Gallstones (Cholelithiasis)
IgG4 Cholangitis
Mirizzi Syndrome
Pancreatic Necrosis and Pancreatic Abscess
Papillary Tumors
Primary Biliary Cholangitis (Primary Biliary Cirrhosis)

Workup

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Media Gallery

Periductal onion skin fibrosis seen in primary sclerosing cholangitis.
Endoscopic retrograde cholangiopancreatography performed in a patient with abnormal liver function test results shows multiple intrahepatic bile duct strictures and beading.
Double-contrast barium enema (same patient as in the previous image) shows filiform polyps and an ahaustral colon resulting from ulcerative colitis.
Percutaneous transhepatic cholangiogram shows dilatation, stricturing, and beading of the intrahepatic bile ducts. Note the surgical clips from a previous cholecystectomy.
This T-tube cholangiogram shows an irregularity of the common bile duct, stricturing, beading, and dilatation of the intrahepatic bile ducts. Note a calculus in the termination of the left hepatic duct (arrow).
Magnetic resonance cholangiopancreatography shows a normal-sized common bile duct, but strictures of both the left and right ducts are noted as well as a dilated proximal left hepatic duct.
This technetium-99m iminodiacetic acid scan shows retention of the radionuclide proximal to strictures in the distribution of the left hepatic duct. Note the lack of filling of the gallbladder because of a previous cholecystectomy. Isotope has entered the small bowel.

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Author

Vikas Khurana, MD, FACP, FACG Assistant Professor, Department of Medicine, Division of Gastroenterology and Hepatology, Graduate Hospital, Gastroenterology Associates, PC

Disclosure: Nothing to disclose.

Coauthor(s)

Tejinder Singh, MD Lead Physician, Section of Emergency Services, Overton Brooks Veterans Affairs Medical Center

Disclosure: Nothing to disclose.

Praveen K Roy, MD, AGAF Clinical Assistant Professor of Medicine, University of New Mexico School of Medicine

Praveen K Roy, MD, AGAF is a member of the following medical societies: American Gastroenterological Association, American Society for Gastrointestinal Endoscopy

Disclosure: Nothing to disclose.

Hisham Nazer, MBBCh, FRCP, DTM&H Professor of Pediatrics, Consultant in Pediatric Gastroenterology, Hepatology and Clinical Nutrition, University of Jordan Faculty of Medicine, Jordan

Hisham Nazer, MBBCh, FRCP, DTM&H is a member of the following medical societies: American Association for Physician Leadership, Royal College of Paediatrics and Child Health, Royal College of Surgeons in Ireland, Royal Society of Tropical Medicine and Hygiene, Royal College of Physicians and Surgeons of the United Kingdom

Disclosure: Nothing to disclose.

Chief Editor

Vinay Kumar Kapoor, MBBS, MS, FRCS, FAMS Professor of Surgical Gastroenterology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, India

Vinay Kumar Kapoor, MBBS, MS, FRCS, FAMS is a member of the following medical societies: Association of Surgeons of India, Indian Association of Surgical Gastroenterology, Indian Society of Gastroenterology, Medical Council of India, National Academy of Medical Sciences (India), Royal College of Surgeons of Edinburgh

Disclosure: Nothing to disclose.

Acknowledgements

Simmy Bank, MD Chair, Professor, Department of Internal Medicine, Division of Gastroenterology, Long Island Jewish Hospital, Albert Einstein College of Medicine

Disclosure: Nothing to disclose.

David Greenwald, MD Associate Professor of Clinical Medicine, Fellowship Program Director, Department of Medicine, Division of Gastroenterology, Montefiore Medical Center, Albert Einstein College of Medicine

David Greenwald, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Gastroenterology, American College of Physicians, American Gastroenterological Association, American Society for Gastrointestinal Endoscopy, and New York Society for Gastrointestinal Endoscopy

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

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