K83.01 Primary sclerosing cholangitis

ICD-10-CM K83.01: Primary Sclerosing Cholangitis

ICD-10-CM code K83.01 represents primary sclerosing cholangitis, an active diagnosis classified under the parent concept K83.0 for cholangitis. The condition is a chronic, progressive cholestatic liver disease of unknown etiology characterized by inflammation and fibrosis of the intrahepatic and extrahepatic bile ducts. 1 2

Plain-language overview

Primary sclerosing cholangitis is a chronic and progressive liver disease marked by inflammation and fibrosis of the intrahepatic and extrahepatic bile ducts, which leads to multifocal strictures and eventual biliary cirrhosis. 2

Despite sharing similar names and certain characteristics, primary sclerosing cholangitis and primary biliary cholangitis are distinct bile duct diseases that necessitate different treatments and monitoring approaches. 3

What this code represents

The ICD-10-CM code K83.01 is an active terminology record representing the disease or syndrome primary sclerosing cholangitis, which is a narrower concept under the parent code K83.0 for cholangitis. 1

The clinical concept for K83.01 denotes a liver disease of unknown etiology, and the terminology record indicates that this specific code has narrower concepts within the classification system. 1 2

Documentation considerations

Clinical documentation for this condition involves identifying the epidemiology, applying current diagnostic criteria while excluding secondary causes of sclerosing cholangitis, and selecting appropriate management approaches for the patient. 2

Evaluating serum IgG4 levels is a relevant clinical consideration, because significantly elevated levels may raise suspicion for IgG4-related sclerosing cholangitis rather than the primary form of the disease. 2

What the sources add

While the terminology record establishes the official code hierarchy and active status, the clinical reference provides detailed disease mechanisms, including the classification into classic large-duct, small-duct, and autoimmune hepatitis overlap subtypes. 1 2

A clinical ontology source describes this condition as a rare and slowly progressive liver disease, whereas another clinical reference emphasizes distinguishing it from primary biliary cholangitis despite their shared naming characteristics. 4 3

Questions to verify

The supplied sources do not establish specific payer coverage policies, reimbursement guidelines, or mandatory clinical documentation elements required to bill the K83.01 diagnosis code for insurance claims. 1 2

Sources

1. ICD-10-CM K83.01 terminology record — National Cancer Institute Enterprise Vocabulary Services; accessed 2026-07-27.
2. Bookshelf — ncbi.nlm.nih.gov; accessed 2026-07-27.
3. PSC PBC Differences | PBC & PSC Similarities | PSC Partners — pscpartners.org; accessed 2026-07-27.
4. Ontology Lookup Service (OLS) — purl.obolibrary.org; accessed 2026-07-27.