E83.01 ICD-10-CM Code: Wilson's disease
E83.01 maps to CMS-HCC V28 50. A source-labeled RAF reference is available. Confirm the documented diagnosis and applicable coding requirements. MEAT criteria · RAF Calculator · HCC coding software
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FY 2026 Apr update / Endocrine, nutritional and metabolic diseases (E00-E89) / Metabolic disorders (E70-E88)
E83.01
Billable / SpecificICD-10-CMOfficial ICD-10-CMCodebook guidanceWilson's disease
A rare inherited disorder where copper accumulates in the liver, brain, and other organs, causing neurological problems, liver disease, and eye abnormalities.
Buddy Insight
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.
CMS-HCC V28
MappedHCC 50
Coefficient HCC 50: 0.648 (Community Non-Dual Aged (CNA))
CMS-HCC V24
HistoricalHistorical
Not used for CY2026 payment
ACA/HHS
MappedHCC 029
Code-level coefficient reference
ESRD/PACE
N/A—
Not mapped
RXHCC
MappedHCC 42
Code-level coefficient reference
Code Book Path
Inclusion Terms
OfficialNo inclusion terms are included in this display for E83.01. Check the code and parent instructions in the Code Book.
Excludes 2
Official- Ehlers-Danlos syndromes (Q79.6-)Inherited from E70-E88
Related Codes
Includes
OfficialNo Includes notes are included in this display for E83.01. Check the code and parent instructions in the Code Book.
Excludes 1
Official- transitory endocrine and metabolic disorders specific to newborn (P70-P74)Inherited from E00-E89, E70-E88, E83
- androgen insensitivity syndrome (E34.5-)Inherited from E00-E89, E70-E88, E83
- congenital adrenal hyperplasia (E25.0)Inherited from E00-E89, E70-E88, E83
- hemolytic anemias attributable to enzyme disorders (D55.-)Inherited from E00-E89, E70-E88, E83
- Marfan syndrome (Q87.4-)Inherited from E00-E89, E70-E88, E83
- 5-alpha-reductase deficiency (E29.1)Inherited from E00-E89, E70-E88, E83
- dietary mineral deficiency (E58-E61)Inherited from E00-E89, E70-E88, E83
- parathyroid disorders (E20-E21)Inherited from E00-E89, E70-E88, E83
- vitamin D deficiency (E55.-)Inherited from E00-E89, E70-E88, E83
Code First
OfficialNo Code First sequencing instructions are included in this display for E83.01. Check the code and parent instructions in the Code Book.
Use Additional
OfficialNo Use Additional Code instructions are included in this display for E83.01. Check the code and parent instructions in the Code Book.
Code Also
Official- associated Kayser Fleischer ring (H18.04-)
Buddy Documentation Tip
MEAT Support
Audit Caution
Common Mistakes
Current with CMS: FY2026 ICD-10-CM Apr 1 update (effective Apr 1 – Sep 30, 2026) · CMS-HCC V28, 100% phased in for payment year 2026. FY2027 code set already staged for October 1, 2026. How HCC Buddy stays current →
Is E83.01 an HCC code?
Yes. E83.01 (Wilson's disease) maps to HCC 50, Amyloidosis, Porphyria, and Other Specified Metabolic Disorders under the CMS-HCC V28 risk adjustment model, with a source-labeled community non-dual aged reference coefficient of 0.648. Source-labeled code-level coefficients are references, not member totals. Actual contribution depends on complete member context, hierarchy and cleanup rules, interactions, and model year. HCC Buddy's RAF Calculator supports CMS-HCC V28 PY2026 and shows no score unless every required source and calculation check passes. It is billable for payment year 2026.
Coder answer: E83.01 is billable and maps to V28 HCC 50, Amyloidosis, Porphyria, and Other Specified Metabolic Disorders. Open it in the Code Book for the tabular path, RAF, and MEAT checklist.
- Code
- E83.01
- Description
- Wilson's disease
- HCC (V28)
- HCC 50 — Amyloidosis, Porphyria, and Other Specified Metabolic Disorders
- RAF reference coefficient
- 0.648
- Billable
- Yes
- Payment year
- 2026
HCC Category Mapping
These are source-labeled model coefficients, not member totals. A category may still be removed by hierarchy or model cleanup rules. Weights are not directly comparable across models: CMS-HCC V28 and V24 use Community, Non-Dual, Aged; ESRD uses the dialysis continuing-enrollee model; RxHCC is the Part D continuing-enrollee, non-low-income, aged weight (a larger scale than CMS-HCC). ACA/HHS has no single weight — it varies by metal level. Actual per-patient RAF contribution depends on member context, hierarchy and cleanup rules, interactions, and the model year used by the payer. V28 is the CMS-HCC model phased in over payment years 2024–2026; V24 remains available for historical review.
Work E83.01 in the Code Book — tabular path, V28 RAF reference, and MEAT checklist →
MEAT review for E83.01
For E83.01, confirm that the documentation supports the diagnosis and meets the applicable coding, encounter, program and payer requirements. MEAT (Monitor, Evaluate, Assess, or Treat) is a review mnemonic, not a universal CMS coding rule.
- MMonitor: signs, symptoms, disease progression, or lab trending documented in the note
- EEvaluate: test results, medication response, or physical findings reviewed by the provider
- AAssess: explicit mention in the assessment or plan with acknowledgment of status
- TTreat: medication, referral, procedure, therapy, or counseling tied to the diagnosis
Coder workflow notes
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What This Code Means
E83.01 is the ICD-10-CM diagnosis code for wilson's disease. A rare inherited disorder where copper accumulates in the liver, brain, and other organs, causing neurological problems, liver disease, and eye abnormalities. E83.01 sits in the ICD-10-CM chapter for endocrine, nutritional and metabolic diseases (e00-e89), within the section covering metabolic disorders (e70-e88).
Under the CMS-HCC V28 risk adjustment model, E83.01 maps to Amyloidosis, Porphyria, and Other Specified Metabolic Disorders (HCC 50) with a source-labeled community, non-dual, aged reference coefficient of 0.648. For CY2026 non-PACE Medicare Advantage, CMS uses 100% of the 2024 CMS-HCC model (V28). PACE uses a separate model blend. Source-labeled code-level coefficients are references, not member totals. Actual contribution depends on complete member context, hierarchy and cleanup rules, interactions, and model year. HCC Buddy's RAF Calculator supports CMS-HCC V28 PY2026 and shows no score unless every required source and calculation check passes.
Wilson's disease is a serious condition requiring specific treatment; ensure accurate diagnosis documentation. For E83.01, confirm that the documentation supports the diagnosis and meets the applicable coding, encounter, program and payer requirements. MEAT (Monitor, Evaluate, Assess, or Treat) is a review mnemonic, not a universal CMS coding rule. When documentation is ambiguous, coders should issue a provider query rather than assume the highest-specificity variant.
HCC Buddy maintains structured V28 and V24 mapping, source-labeled coefficient references, and MEAT documentation criteria for E83.01 sourced directly from the CMS-HCC risk adjustment model files and the CMS ICD-10-CM code set.
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