K74.3
BillablePrimary biliary cirrhosis
HCC Category Mapping
V28HCC 68 — Cholangitis and Biliary Cirrhosis
0.000V24HCC 28 — Cirrhosis of Liver
0.390ESRDHCC 28 — Cirrhosis of Liver
0.000RxHCCHCC 59 — Primary Biliary Cirrhosis/Cholangitis
0.000What This Code Means
A type of cirrhosis affecting the bile ducts that is caused by autoimmune disease, leading to progressive liver damage.
Coding Tips
- •Primary biliary cirrhosis is now often called primary biliary cholangitis (PBC); both terms refer to the same condition
- •Confirm autoimmune etiology in documentation to differentiate from secondary biliary cirrhosis (K74.4)
Clinical Significance
Primary biliary cirrhosis is an autoimmune condition causing progressive destruction of bile ducts, leading to cirrhosis and potential liver failure. This diagnosis requires lifelong monitoring and specialized treatment, significantly impacting patient care complexity and resource utilization.
Documentation Requirements
- ✓Positive antimitochondrial antibodies (AMA) or specific antinuclear antibodies
- ✓Elevated alkaline phosphatase levels
- ✓Liver biopsy showing bile duct destruction and portal inflammation
- ✓Clinical presentation with fatigue, pruritus, or jaundice
- ✓Exclusion of other causes of biliary cirrhosis
- ✓Imaging showing biliary tract abnormalities
- ✓Documentation of progressive cholestatic liver disease
- ✓Evidence of autoimmune etiology
Excludes 2 — Not included here, may code separately
- primary sclerosing cholangitis (K83.01)
Commonly Confused Codes
K74.4 — Secondary biliary cirrhosis caused by obstruction rather than autoimmuneK74.5 — Biliary cirrhosis unspecified when primary vs secondary not documentedK83.01 — Primary sclerosing cholangitis affects larger bile ductsK75.4 — Autoimmune hepatitis affects hepatocytes rather than bile ductsK74.60 — Unspecified cirrhosis when biliary nature not established