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E83.01 ICD-10-CM Code: Wilson's disease

Billing Status: YES. This is a valid, specific, and billable ICD-10-CM reference.

Coding at a Glance

Tabular directives
8 Excludes1 · 1 Excludes2 · 1 code-also instruction
Risk adjustment
CMS-HCC V28: 1 category · RxHCC V08 category 42

Inpatient Payment Groups (MS-DRG)

Potential MS-DRG participation — not a DRG assignment.

MS-DRGs this diagnosis helps define, as principal or secondary, per the CMS ICD-10-CM/PCS MS-DRG Definitions Manual v43, Appendix B.

  • MS-DRG 642 — INBORN AND OTHER DISORDERS OF METABOLISM (MDC 10)

A diagnosis appearing in a group's logic does not by itself determine the DRG assigned to a stay; the grouper uses the full claim.

Risk Adjustment (CMS-HCC)

Payment categories this diagnosis maps to under CMS-HCC V28, payment year 2026.

  • HCC 50 — Amyloidosis, Porphyria, and Other Specified Metabolic Disorders

Other models: RxHCC V08 HCC 42

Risk scores depend on the enrollee's full accepted diagnosis set and segment; a category mapping alone does not determine payment.

Coding instructions

Official Tabular Instructional Notes: the inclusion, exclusion and sequencing notes published for E83.01 in the official ICD-10-CM tabular list, quoted as published.

Source: CMS/CDC — ICD-10-CM Tabular ListRelease: FY2026Effective: October 1, 2025

Notes without a marker are published on E83.01 itself; “inherited from” names the category or block whose note applies here.

Excludes1 — Not Coded Here

Conditions generally not reported together with this code (Excludes1) -- an error unless the two conditions are documented as unrelated to each other (ICD-10-CM Official Guidelines, Section I.A.12.a).

Excludes2 — Not Included Here

Conditions not covered by this code, but which may be reported alongside it when both are present.

Source: inherited from E70-E88

Code Also

Additional codes that may be required to fully describe the encounter.

  • associated Kayser Fleischer ring (H18.04-)

Coder workflow for E83.01

MedCoder structured workflow — derived from this code’s own official record

Before you code E83.01

  1. Check the Excludes1 note: if the documentation supports a condition named there, do not simply proceed with E83.01. Excludes1 conditions are not reported together with this code unless the record shows they are unrelated (Guidelines I.A.12.a).

    See the official tabular notes

Choose the right path

  1. Does the documentation support a condition named in E83.01’s Excludes1 note?
    Yes → Do not simply proceed: review the excluded code. Both are reported only when the record shows the two conditions are unrelated.
    No → Continue.

    ReviewE55, E34.5, E25.0, D55, Q87.4, E29.1

Consider E83.01. Then review the Code Also note, and confirm the code is valid for the date of service in the Verify section.

Documentation check

The provider’s diagnostic statement
Codes are assigned from the provider’s documented diagnosis, not from clinical criteria, test values or a medication list (Guidelines I.A.19).

Official instructions as workflow

  • Excludes1 — check before selecting E83.01(8 notes)

    Coding workflow: If the documentation supports a condition named in this note, do not simply proceed with E83.01: the two are not reported together. The one exception is when the record shows the two conditions are unrelated to each other.

    CompareE55, E34.5, E25.0, D55, Q87.4, E29.1

    See the official tabular notes · Guidelines I.A.12.a

  • Excludes2 — not part of E83.01(1 note)

    Coding workflow: The conditions named in this note are not included in E83.01. When the record documents both, both may be reported; the note is a boundary, not a prohibition.

    CompareQ79.6

    See the official tabular notes · Guidelines I.A.12.b

  • Code Also — related condition(1 note)

    Coding workflow: Review the related condition when both are documented and the instruction applies. A Code Also note does not fix sequencing; the order follows the circumstances of the encounter.

    ReviewH18.04

    See the official tabular notes · Guidelines I.A.17

Coding decision scenarios

Pattern scenarios for this code’s structure — decision rules, not clinical cases

Documentation: Both the condition E83.01 describes and a condition named in its Excludes1 note are documented for the same encounter.

Coding question: Can both codes be reported?

Path: Review the Excludes1 note and the excluded code, and look for a provider statement on whether the two conditions are related.

Reason: Excludes1 means the two are not coded together; the exception is when the record shows the conditions are unrelated to each other (Guidelines I.A.12.a).

ReviewE55, E34.5, E25.0, D55, Q87.4, E29.1

Every row is derived from this code’s own record — its title, tabular notes, 7th-character family and same-category siblings — with fixed MedCoder wording; nothing is inferred about a patient. The official notes and guideline text are in the sections each row links to, and they control.

Code Overview

Wilson's disease is a billable ICD-10-CM diagnosis code (E83.01).

MedCoder summary Summary composed by MedCoder from this code's official ICD-10-CM record. The tabular instructional notes themselves appear verbatim below.

Indexed Clinical Terms (26)

Official source data — entries quoted as published, in the Index’s own lookup phrasing

Clinical term phrases from the official ICD-10-CM Index to Diseases and Injuries that map to this code. These are alphabetic-index entries shown as the Index writes them — lookup phrasing, not necessarily the wording of a final diagnosis.

Decision Points

The directives on this code's own record, as a pre-claim checklist.

  1. 1 Code Also note — a second code may apply; the guidelines leave its sequencing to the circumstances of the encounter. See the Code Also notes
  2. 8 Excludes1 entries — codes named there are generally not reported together with this code (Guidelines I.A.12.a). See the Excludes1 notes
  3. 1 Excludes2 entry — those conditions are not part of this code and may be reported additionally when documented. See the Excludes2 notes

Checklist rows are derived from this code's own official directives; the wording of each check is MedCoder editorial. The official notes themselves are in the sections each row links to.

Verify Before Coding

  • No Medicare Code Editor or MS-DRG Definitions Manual restrictions apply to this code.

From the code registry, the Medicare Code Editor, and the MS-DRG Definitions Manual. Check it against a full claim in Claim Check.

Relationships & Classification

MedCoder structured relationships — computed from published CMS and AHRQ datasets

Other codes that name E83.01 or its code family, from the CMS ICD-10-CM tabular instructional notes. Tabular-note edges are stored at the code family level that carries each note.

Referenced by 3 Excludes1 notes across 3 chapters: E61 — Deficiency of other nutrient elements (via E83.-), P59 — Neonatal jaundice from other and unspecified causes (via E83.-), R79.0 — Abnormal level of blood mineral (via E83.-).

These codes’ tabular lists mark this diagnosis as mutually exclusive — not reported together unless the conditions are documented as unrelated.

Referenced by 13 Excludes2 notes across 3 chapters: G11 — Hereditary ataxia (via E83.-), G71 — Primary disorders of muscles (via E83.-), K70 — Alcoholic liver disease, K70-K77 — Diseases of liver (K70-K77), K71 — Toxic liver disease, K72 — Hepatic failure, not elsewhere classified, K73 — Chronic hepatitis, not elsewhere classified, K74 — Fibrosis and cirrhosis of liver, K75 — Other inflammatory liver diseases, K76 — Other diseases of liver, K77 — Liver disorders in diseases classified elsewhere, N25.0 — Renal osteodystrophy (via E83.-), N25.81 — Secondary hyperparathyroidism of renal origin (via E83.-).

These codes’ tabular lists name this diagnosis as distinct — both may be reported when both are documented.

Referenced by 4 Code First instructions across 4 chapters: F02 — Dementia in other diseases classified elsewhere, G63 — Polyneuropathy in diseases classified elsewhere (via E83.-), H42 — Glaucoma in diseases classified elsewhere (via E83.-), N16 — Renal tubulo-interstitial disorders in diseases classified elsewhere.

Each of these codes carries a Code First note naming this condition — when that code is reported, THIS code is sequenced first, ahead of it.

Referenced by 1 Code Also instruction: H18.04 — Kayser-Fleischer ring.

These codes suggest coding this condition alongside when both are present.

MS-DRG Grouper Relationships (FY2026)

Potential MS-DRG participation — not a DRG assignment.

FY2026 MS-DRG: not on the CMS CC/MCC list — as a secondary diagnosis this code does not change MS-DRG severity for that release.

Named in the grouper logic of 1 MS-DRG: DRG 642 (MDC 10).

From the CMS MS-DRG Definitions Manual (Appendices B and C). Actual DRG assignment depends on the complete claim.

Clinical classification (AHRQ CCSR):END016 — Other specified and unspecified nutritional and metabolic disorders (default).

Clinical Classifications Software Refined (CCSR) for ICD-10-CM Diagnoses. Healthcare Cost and Utilization Project (HCUP), Agency for Healthcare Research and Quality.

Related Codes

Same CMS-HCC risk category (V28)

CMS maps these diagnoses to the same Hierarchical Condition Category (Amyloidosis, Porphyria, and Other Specified Metabolic Disorders) for risk-adjusted payment.

E74.05 — Lysosome-associated membrane protein 2 [LAMP2] deficiency, E74.09 — Other glycogen storage disease, E79.1 — Lesch-Nyhan syndrome, E80.0 — Hereditary erythropoietic porphyria, E80.1 — Porphyria cutanea tarda, E80.20 — Unspecified porphyria, E80.21 — Acute intermittent (hepatic) porphyria, E80.29 — Other porphyria, E80.3 — Defects of catalase and peroxidase, E83.00 — Disorder of copper metabolism, unspecified, E83.09 — Other disorders of copper metabolism, E83.31 — Familial hypophosphatemia, E85.0 — Non-neuropathic heredofamilial amyloidosis, E85.1 — Neuropathic heredofamilial amyloidosis, E85.2 — Heredofamilial amyloidosis, unspecified, E85.3 — Secondary systemic amyloidosis, E85.4 — Organ-limited amyloidosis, E85.81 — Light chain (AL) amyloidosis, E85.82 — Wild-type transthyretin-related (ATTR) amyloidosis, E85.89 — Other amyloidosis, +11 more

Same clinical category (CCSR)

AHRQ's Clinical Classifications Software groups these diagnoses under the same clinical category (Other specified and unspecified nutritional and metabolic disorders).

E80.1 — Porphyria cutanea tarda, E80.20 — Unspecified porphyria, E80.21 — Acute intermittent (hepatic) porphyria, E80.29 — Other porphyria, E80.3 — Defects of catalase and peroxidase, E80.4 — Gilbert syndrome, E80.5 — Crigler-Najjar syndrome, E80.6 — Other disorders of bilirubin metabolism, E80.7 — Disorder of bilirubin metabolism, unspecified, E83.00 — Disorder of copper metabolism, unspecified, E83.09 — Other disorders of copper metabolism, E83.10 — Disorder of iron metabolism, unspecified, E83.110 — Hereditary hemochromatosis, E83.111 — Hemochromatosis due to repeated red blood cell transfusions, E83.118 — Other hemochromatosis, E83.119 — Hemochromatosis, unspecified, E83.19 — Other disorders of iron metabolism, E83.2 — Disorders of zinc metabolism, E83.30 — Disorder of phosphorus metabolism, unspecified, E83.31 — Familial hypophosphatemia, +258 more

Same Index main term, other category

The ICD-10-CM Index to Diseases and Injuries files this code under the main terms “Degeneration, degenerative”, “Deficiency, deficient”, “Dementia”, …; these codes share that main term but sit in a different category of the Tabular List.

E75.3 — Sphingolipidosis, unspecified (brain, in, sphingolipidosis), E75.4 — Neuronal ceroid lipofuscinosis (brain, in, lipidosis, cerebral), E75.6 — Lipid storage disorder, unspecified (brain, in, lipidosis, generalized), E76.1 — Mucopolysaccharidosis, type II (brain, in, Hunter's syndrome), E76.29 — Other mucopolysaccharidoses (beta-glucuronidase), E78.2 — Mixed hyperlipidemia (due to, xanthomatosis), E78.6 — Lipoprotein deficiency (lipoprotein), E79.1 — Lesch-Nyhan syndrome (hypoxanthine-), E79.2 — Myoadenylate deaminase deficiency (myoadenylate deaminase), E80.5 — Crigler-Najjar syndrome (glucuronyl transferase), E85.4 — Organ-limited amyloidosis (skin, amyloid), E85.89 — Other amyloidosis (capillaries, amyloid), E85.9 — Amyloidosis, unspecified (amyloid), E87.1 — Hypo-osmolality and hyponatremia (salt), E87.6 — Hypokalemia (kalium), E88.01 — Alpha-1-antitrypsin deficiency (alpha-1-antitrypsin), E88.02 — Plasminogen deficiency (plasminogen), E88.09 — Other disorders of plasma-protein metabolism, not elsewhere classified (pseudocholinesterase), E88.89 — Other specified metabolic disorders (dihydropyrimidine dehydrogenase), F01.50 — Vascular dementia, unspecified severity, without behavioral disturbance, psychotic disturbance, mood disturbance, and anxiety (vascular), +437 more

Lab tests where this diagnosis supports Medicare coverage (NCD)

Medicare's National Coverage Determination (NCD) program lists this diagnosis as medical justification for these lab tests.

Alpha-Fetoprotein (AFP) Test, Gamma-glutamyl Transferase (GGT) Test, Partial Thromboplastin Time (PTT) Test

Contextual Map

Every relationship of E83.01 in one view: hierarchy, official tabular instructions in both directions, clinical classification, risk adjustment, MS-DRG participation, index terms and change history — each edge carrying the CMS source it derives from.

Run E83.01 with these 16 related codes in Claim Check

Hierarchy

Code Also

Referenced by Excludes1 notes

Referenced by Excludes2 notes (13)

Referenced by Code First instructions

Referenced by Code Also instructions

Clinical classification (CCSR)

Risk adjustment (CMS-HCC)

  • HCC 50 — Amyloidosis, Porphyria, and Other Specified Metabolic Disorders [CMS-HCC]— CMS-HCC V28 · 2026

MS-DRG Grouper

MDC crossing

  • MDC 10 — Endocrine, Nutritional and Metabolic Diseases and Disorders[MDC crossing]: “Endocrine, Nutritional and Metabolic Diseases and Disorders — the grouper's crossing between diagnoses and procedures: a principal diagnosis sets the MDC, and same-MDC procedures move the stay to its surgical DRGs. 5,008 same-MDC procedures group here; browse them on the MDC page.”— CMS MS-DRG Definitions Manual · FY2026

Index entries (26)

  • Cirrhosis, cirrhotic (hepatic) (liver), due to, hepatolenticular degeneration[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2026
  • Cirrhosis, cirrhotic (hepatic) (liver), due to, Wilson's disease[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2026
  • Deficiency, deficient, ceruloplasmin (Wilson)[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2026
  • Degeneration, degenerative, hepatolenticular (Wilson's)[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2026
  • Degeneration, degenerative, lenticular (familial) (progressive) (Wilson's) (with cirrhosis of liver)[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2026
  • Degeneration, degenerative, Wilson's hepatolenticular[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2026
  • Dementia (degenerative (primary)) (persisting) (unspecified severity) (without behavioral disturbance, psychotic disturbance, mood disturbance, and anxiety), in (due to), hepatolenticular degeneration[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2026
  • Dementia (degenerative (primary)) (persisting) (unspecified severity) (without behavioral disturbance, psychotic disturbance, mood disturbance, and anxiety), in (due to), hepatolenticular degeneration, with behavioral disturbance[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2026
  • and 18 more

Nearest codes (39)

Change history

  • FY2016 — In the code set at ICD-10-CM adoption [Change history]— CMS release files (code change ledger) · icd10cm-fy2016

Sources for this page

Codes, titles, notes, index terms and mappings on this page are transcribed from the datasets below. Relationships MedCoder computed and text MedCoder wrote are labelled where they appear.

Code, title, tabular notes and index terms Official source data
CMS/CDC ICD-10-CM FY2026 tabular list, index and tables, effective October 1, 2025 Release, file and checksum · Publisher’s page
Claim edits Official source data
CMS Definitions of Medicare Code Edits — v43.1 (April 2026) Release, file and checksum · Publisher’s page
Inpatient payment groups Official source data
CMS MS-DRG Definitions Manual (incl. Appendix B diagnosis index, Appendix C CC/MCC list, Appendix E procedure index) and IPPS Final Rule tables — v43 Release, file and checksum · Publisher’s page
Risk adjustment Official source data
2026 Mid-Year Final ICD-10 Mappings + Model Software (cms.gov/files/zip/2026-midyear-final-icd-10-mappings.zip, 2026-midyear-final-model-software.zip) — CMS-HCC V28, PY2026 mid-year final release Release, file and checksum · Publisher’s page
Change history and date-of-service validity Official source data
CMS ICD-10-CM release addenda, ingested release by release into the change ledger Release, file and checksum · Publisher’s page
Comparisons, relationships and the contextual map MedCoder structured relationship
Computed by MedCoder from the tabular notes and tables above; every derived relationship is marked as derived where it appears
Summary and FAQ answers MedCoder explanation
Written by MedCoder to explain the sources above: drafted with AI assistance, checked by a person against the release files, and labelled as MedCoder text where it appears. Not official text.

Labels on this page: Official source data · MedCoder structured relationship · MedCoder explanation. How to read the labels · All data sources and release dates

Cite this page

Reference this page in a research guide, syllabus or article. The release is included because code content changes each year.

MedCoder.ai. "E83.01 — Wilson's disease." ICD-10-CM FY2026. https://medcoder.ai/icd10/code/e83.01-wilsons-disease

Change history

  • FY2016 — October 1, 2015
    In the code set at ICD-10-CM adoption
    Wilson's disease

No changes since FY2016 — additions, deletions, description changes and billable-status changes are tracked through FY2027 (effective October 1, 2026), and none are recorded for this code. Note changes are tracked from FY2027 only.

Nearest Codes in This Family

Official ICD-10-CM classifications closest to E83.01 in its code family, with their registry titles.

View all codes in the E83 family