E83.01

Billable

Wilson's disease

HCC Category Mapping

V28HCC 50 — Glycogen/Amino-Acid/Other Metabolic Disorders

0.289

RxHCCHCC 42 — Endocrine Disorders and Metabolic Conditions

0.000

What This Code Means

A rare inherited disorder where copper accumulates in the liver, brain, and other organs, causing neurological problems, liver disease, and eye abnormalities.

Coding Tips

•Wilson's disease is a serious condition requiring specific treatment; ensure accurate diagnosis documentation
•Code any associated complications separately (cirrhosis, neurological manifestations, Kayser-Fleischer rings)

Clinical Significance

Wilson disease is an autosomal recessive disorder of copper metabolism caused by ATP7B gene mutations, leading to toxic copper accumulation in the liver, brain, and other organs. Without treatment, it is fatal, but early diagnosis and lifelong chelation therapy or zinc supplementation can be life-saving. Liver transplantation is curative for hepatic Wilson disease.

Documentation Requirements

✓Provider diagnosis of Wilson disease
✓Confirmatory diagnostic criteria: low serum ceruloplasmin, elevated 24-hour urine copper, hepatic copper on liver biopsy, Kayser-Fleischer rings on slit-lamp exam
✓ATP7B gene mutation analysis if performed
✓Hepatic manifestations: liver function tests, imaging, fibrosis staging
✓Neurological/psychiatric manifestations: tremor, dysarthria, dystonia, personality changes
✓Current treatment: D-penicillamine, trientine, zinc acetate, or liver transplant status
✓Treatment compliance monitoring: urine copper, serum copper levels

Code Also

associated Kayser Fleischer ring (H18.04-)

Commonly Confused Codes

E83.00 — Disorder of copper metabolism, unspecified: use only when Wilson disease is not specifically confirmed E83.09 — Other disorders of copper metabolism: for copper disorders that are NOT Wilson disease (e.g., Menkes disease in some classifications)K74.69 — Other cirrhosis of liver: Wilson disease can present as cirrhosis, but the metabolic code is primary G25.9 — Extrapyramidal and movement disorder, unspecified: neurological Wilson may mimic other movement disorders

Code Hierarchy

└E83Disorders of mineral metabolism └E83.0Disorders of copper metabolism └E83.01Wilson's disease

└E83.01Wilson's disease