ICD-10-CM 2027 diagnosis code
E83.01Wilson's disease
E83.01 is a valid, billable ICD-10-CM code for wilson's disease. It can be reported on claims for dates of service from October 1, 2026 – September 30, 2027.
Looking up by condition? See ICD-10 code for Westphal-Strümpell syndrome, ICD-10 code for Hepatolenticular degeneration, ICD-10 code for Strümpell-Westphal pseudosclerosis.
Code unchanged since ICD-10-CM took effect on October 1, 2015. Source: official FY2027 ICD-10-CM release from CDC/NCHS and CMS. Page updated September 29, 2026. About our data
About E83.01
E83.01 is the ICD-10-CM diagnosis code for Wilson's disease. It belongs to category E83 (disorders of mineral metabolism), block E70-E88 (metabolic disorders) and chapter 4 (endocrine, nutritional and metabolic diseases). It is billable, so it can be reported on its own for dates of service from October 1, 2026 through September 30, 2027.
Within E83.0 (disorders of copper metabolism), E83.01 is specifically for Wilson's disease. Related codes cover disorder of copper metabolism, unspecified (E83.00) and other disorders of copper metabolism (E83.09).
It is not a CC or MCC, so as a secondary diagnosis it doesn't change the MS-DRG severity level.
In MS-DRG v44.0, it is part of the grouping logic for DRG 642 (Inborn and Other Disorders of Metabolism, relative weight 1.3219), in MDC 10 (Endocrine, Nutritional and Metabolic Diseases and Disorders), as a principal or secondary diagnosis. The DRG a claim lands in depends on the principal diagnosis, procedures and any CC/MCC secondary diagnoses.
It has been part of ICD-10-CM since the code set took effect on October 1, 2015 and hasn't changed since.
Before ICD-10, this condition was coded in ICD-9-CM as 275.1 (disorders of copper metabolism).
Coding notes
- associated Kayser Fleischer ring (H18.04-)
Notes that apply from higher levels
Instructions written at a parent level also apply to E83.01.
Broader instructions also apply from E70-E88 Metabolic disorders and Chapter 4: Endocrine, nutritional and metabolic diseases.
Alphabetic index entries
26 entriesTerms in the official ICD-10-CM index that lead to E83.01.
- Cirrhosis, cirrhotic (hepatic) (liver) › due to › hepatolenticular degeneration
- Cirrhosis, cirrhotic (hepatic) (liver) › due to › Wilson's disease
- Deficiency, deficient › ceruloplasmin (Wilson)
- Degeneration, degenerative › hepatolenticular (Wilson's)
- Degeneration, degenerative › lenticular (familial) (progressive) (Wilson's) (with cirrhosis of liver)
- Degeneration, degenerative › Wilson's hepatolenticular
- Dementia (degenerative (primary)) (persisting) (unspecified severity) (without behavioral disturbance, psychotic disturbance, mood disturbance, and anxiety) › in (due to) › hepatolenticular degeneration
- Dementia (degenerative (primary)) (persisting) (unspecified severity) (without behavioral disturbance, psychotic disturbance, mood disturbance, and anxiety) › in (due to) › hepatolenticular degeneration › with behavioral disturbance
- Disease, diseased › hepatolenticular
- Disease, diseased › Kinnier Wilson's (hepatolenticular degeneration)
- Disease, diseased › Wilson's (hepatolenticular degeneration)
- Disorder (of) › metabolism NOS › copper › Wilson's disease
- Disorder (of) › tubulo-interstitial (in) › Wilson's disease
- Hepatolenticular degeneration
- Kinnier Wilson's disease (hepatolenticular degeneration)
- Lenticular degeneration, progressive
- Pseudosclerosis (brain) › of Westphal (Strümpell)
- Pyelonephritis › in (due to) › Wilson's disease
- Strümpell-Westphal pseudosclerosis
- Syndrome › amyostatic (Wilson's disease)
- Syndrome › lenticular, progressive
- Syndrome › Westphal-Strümpell
- Syndrome › Wilson's (hepatolenticular degeneration)
- Westphal-Strümpell syndrome
- Wilson's › disease or syndrome
- Wilson's › hepatolenticular degeneration
Codes whose notes reference E83.01
MS-DRG v44.0 grouping
All DRGs →Inpatient MS-DRGs this diagnosis can group to, as the principal or a secondary diagnosis, depending on the rest of the claim.
MDC 10 · Endocrine, Nutritional and Metabolic Diseases and Disorders
ICD-9-CM equivalent
Converter →- 275.1Disorders of copper metabolismapproximate
From the CMS 2018 General Equivalence Mappings (GEMs), the final GEMs release.
Code history
- 2016
- 2017
- 2018
- 2019
- 2020
- 2021
- 2022
- 2023
- 2024
- 2025
- 2026
- 2027
- FY2016 (effective 10/1/2015): Added (first year of ICD-10-CM)
- No changes since FY2016.
Common questions about E83.01
Is E83.01 billable?
- Yes. E83.01 is a billable/specific ICD-10-CM code valid for FY2027 (October 1, 2026 – September 30, 2027).
Can E83.01 be used as a principal diagnosis?
- Yes. The Medicare Code Editor doesn't restrict E83.01 as a principal diagnosis.
Is E83.01 a CC or MCC?
- No. E83.01 is neither a CC nor an MCC under MS-DRG v44.0.
What DRG does E83.01 group to?
- E83.01 is used in the MS-DRG v44.0 logic for MS-DRG 642 (Inborn and Other Disorders of Metabolism). Which one applies depends on whether it is the principal diagnosis and on the rest of the claim.
What is the ICD-9 code for E83.01?
- The CMS General Equivalence Mappings map E83.01 to ICD-9-CM 275.1.