2027 ICD-10-CM Diagnosis Code E83.01Wilson's disease
ICD-10-CM Codes›E00–E89›E70-E88›E83
- Billable — Valid for Submission
- Risk Adjusts — HCC 50
- Chronic Condition
E83.01 is a billable ICD-10-CM diagnosis code for Wilson's disease. It is valid on HIPAA claims for fiscal year 2027 (October 1, 2026 through September 30, 2027) and groups to MS-DRG 642. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Other specified and unspecified nutritional and metabolic disorders.
For Medicare Advantage risk adjustment, E83.01 maps to CMS-HCC Category 50 (Amyloidosis, Porphyria, and Other Specified Metabolic Disorders) under the V28 model, adding a risk factor of about 0.648 for a community, non-dual, aged beneficiary in payment year 2027.
Code Identity
Code Classification
Medicare Risk Adjustment (HCC)Billing
E83.01 maps to a payment category in the CMS-HCC model used to risk-adjust Medicare Advantage payments. Weights are the published community factors for payment year 2027.
Source: CMS Payment Year 2027 risk adjustment mappings and model software. Weights are relative factors, not dollar amounts; a beneficiary's total RAF also includes demographics and interactions. Browse all CMS-HCC categories.
Approximate SynonymsGuidance
Alternate terms and clinical phrases that map to this code.
- Chorea co-occurrent and due to Wilson disease
- Chorea due to heredodegenerative disorder
- Chorea due to metabolic disorder
- Dementia due to genetic disease
- Dementia due to metabolic abnormality
- Dementia due to Wilson disease
- Disorder of copper metabolism
- Disorder of liver due to disorder of mineral metabolism
- Dystonia due to Wilson disease
- Hypoparathyroidism due to Wilson disease
- Westphal-Strumpell syndrome
- Wilson's disease
Instructional NotesGuidance
Instructions from the official ICD-10-CM Tabular List that apply to E83.01: its own notes plus those printed at E83, block E70-E88, and Chapter 4. A note printed at a category, block or chapter applies to every code under it.
Excludes1
Not coded here: the excluded code is never reported together with this one, unless the two conditions are unrelated.
Excludes2
Not included here: the excluded condition is not part of this code, but a patient may have both, so both codes may be reported.
- Ehlers-Danlos syndromes (Q79.6-)
Code Also
A second code may be needed to fully describe the condition; the note gives no sequencing direction.
- associated Kayser Fleischer ring (H18.04-)
Notes
General instructions on how these codes are used.
- All neoplasms, whether functionally active or not, are classified in Chapter 2. Appropriate codes in this chapter (i.e. E05.8, E07.0, E16-E31, E34.-) may be used as additional codes to indicate either functional activity by neoplasms and ectopic endocrine tissue or hyperfunction and hypofunction of endocrine glands associated with neoplasms and other conditions classified elsewhere.
Source: CMS ICD-10-CM Tabular List. How to read instructional notes.
Referenced in Other NotesGuidance
Instructional notes printed at other codes that name E83.01, its category, or a range that includes it.
Excludes1 2
These codes carry an Excludes1 note naming E83.01: they are not reported together with it, unless the two conditions are unrelated.
Excludes2 1
These codes carry an Excludes2 note naming E83.01: its condition is not part of those codes, and both may be reported when the patient has both.
- Block K70-K77 Diseases of liverWilson's disease (E83.01)
Code First 2
These codes say to code first a condition named in the note, and E83.01 is one of the conditions named.
Index to Diseases and InjuriesGuidance
Alphabetical index entries that point to this code.
Dementia (degenerative (primary)) (persisting) (unspecified severity) (without behavioral disturbance, psychotic disturbance, mood disturbance, and anxiety) F03.90
hepatolenticular degeneration See Also: Dementia, in, diseases specified elsewhere; E83.01
with behavioral disturbance See Also: Dementia, in, diseases specified elsewhere; E83.01
Disease, diseased See Also: Syndrome;
Disorder (of) See Also: Disease;
Pyelonephritis See Also: Nephritis, tubulo-interstitial;
Wilson's disease E83.01
Syndrome See Also: Disease;
lenticular, progressive E83.01
Westphal-Strümpell E83.01
Westphal-Strümpell syndrome E83.01
disease or syndrome E83.01
Clinical ClassificationClinical
AHRQ’s CCSR groups this code into broader clinical categories.
Clinical InformationClinical
Hepatolenticular Degeneration
a rare autosomal recessive disease characterized by the deposition of copper in the brain; liver; cornea; and other organs. it is caused by defects in the atp7b gene encoding copper-transporting atpase 2 (ec 3.6.3.4), also known as the wilson disease protein. the overload of copper inevitably leads to progressive liver and neurological dysfunction such as liver cirrhosis; tremor; ataxia and intellectual deterioration. hepatic dysfunction may precede neurologic dysfunction by several years.
Patient EducationClinical
Wilson Disease
Wilson disease is a rare inherited disorder that prevents your body from getting rid of extra copper. You need a small amount of copper from food to stay healthy. Too much copper is poisonous.
Read the full article at MedlinePlus
Courtesy of MedlinePlus, a service of the U.S. National Library of Medicine.
Convert E83.01 to ICD-9-CMHistory
The closest ICD-9-CM equivalents under the General Equivalence Mappings.
Code HistoryHistory
Questions About E83.01Overview
What is the ICD-10 code for Wilson's disease?
The ICD-10-CM code for Wilson's disease is E83.01 (sometimes written as E8301). It is billable on HIPAA-covered claims from October 1, 2026 through September 30, 2027.
Is E83.01 (Disorders of copper metabolism) a billable code?
Yes. This is a billable ICD-10-CM code, specific enough to report Wilson's disease on HIPAA-covered claims from October 1, 2026 through September 30, 2027.
What MS-DRG does E83.01 group to?
When Wilson's disease is the principal diagnosis on an inpatient stay, it groups to MS-DRG 642 (inborn and Other Disorders of Metabolism), which carries a relative weight of 1.3226. Higher weights mean higher Medicare reimbursement.
Can E83.01 be reported with a code from E55?
Not as a rule. The Excludes1 note at E83, which applies to E83.01, lists "vitamin D deficiency (E55.-)". An Excludes1 note means the excluded code is never reported together with this one; the only exception is when the two conditions are unrelated to each other (Official Guidelines, Section I.A.12.a).
What is the ICD-9 equivalent of E83.01?
Under the General Equivalence Mappings, Wilson's disease converts to ICD-9-CM 275.1 (dis copper metabolism). The mapping is approximate, so confirm the match fits the documentation.
What HCC is E83.01?
E83.01 (Wilson's disease) maps to CMS-HCC Category 50 (Amyloidosis, Porphyria, and Other Specified Metabolic Disorders), commonly written as HCC 50, in the CMS-HCC V28 model used for Medicare Advantage risk adjustment in payment year 2027. In the Part D prescription drug model it maps to RxHCC 42.
Does E83.01 risk-adjust for Medicare Advantage payment?
Yes. When documented and reported on a Medicare Advantage encounter, E83.01 adds a risk adjustment factor of about 0.648 to the beneficiary's RAF score for a community, non-dual, aged enrollee (published V28 weights range from 0.362 to 0.883 depending on the payment segment). HCC 50 sits at the top of its hierarchy, so no other condition category supersedes it. See the full factor table on the HCC 50 category page.
