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E85.0 ICD-10-CM Code: Non-neuropathic heredofamilial amyloidosis

Compare with another code for E85.0Check this code on a claim

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

Coding at a Glance

Billable · FY2027A valid, specific ICD-10-CM code, reportable for dates of service in FY2027.

What you need to know

Source: CMS/NCHS Official ICD-10-CM tabular notes, quoted. From the CMS/NCHS tabular list for the release in force. A note the category or block publishes applies to this code too, and is marked with where it comes from.

Excludes1from E70-E88Not reported with this code unless documented as unrelated
  • androgen insensitivity syndrome (E34.5-)
  • congenital adrenal hyperplasia (E25.0)
  • hemolytic anemias attributable to enzyme disorders (D55.-)
  • Marfan syndrome (Q87.4-)

+1 more in Instructions

Code alsoMay be needed with this code
  • associated disorders, such as:
  • autoinflammatory syndromes (M04.-)
Excludes2Not included here; may be reported together
  • Transthyretin-related (ATTR) familial amyloid cardiomyopathy (E85.4)
  • Alzheimer's disease (G30.0-) (from E85)
  • Ehlers-Danlos syndromes (Q79.6-) (from E70-E88)
IncludesWhat this code covers
  • Hereditary amyloid nephropathy

Most relevant related codes MedCoder-derived

Read off the official notes above and this code’s own position in the tabular list. Which to report is a documentation question; Compare shows the two side by side.

CompareCheck ClaimView Related Codes

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 v44, Appendix B.

  • MS-DRG 545 — CONNECTIVE TISSUE DISORDERS WITH MCC (MDC 08)
  • MS-DRG 546 — CONNECTIVE TISSUE DISORDERS WITH CC (MDC 08)
  • MS-DRG 547 — CONNECTIVE TISSUE DISORDERS WITHOUT CC/MCC (MDC 08)

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: CMS-HCC V22 HCC 23

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 E85.0 in the official ICD-10-CM tabular list, quoted as published.

Source: CMS/CDC — ICD-10-CM Tabular ListRelease: FY2027Effective: October 1, 2026

Trace:FY2027 changesChange historyRelease, file and checksum

Notes without a marker are published on E85.0 itself; “inherited from” names the category or block whose note applies here.

Inclusion Terms

Alternative terms the tabular list files under this code.

  • Hereditary amyloid nephropathy

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).

Source: inherited from E70-E88

Excludes2 — Not Included Here

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

Code Also

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

  • associated disorders, such as:
  • autoinflammatory syndromes (M04.-)

Coder workflow for E85.0

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

Before you code E85.0

  1. Check the Excludes1 note: if the documentation supports a condition named there, do not simply proceed with E85.0. 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 E85.0’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.

    ReviewE34.5, E25.0, D55, Q87.4, E29.1

Consider E85.0. 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 E85.0(5 notes)

    Coding workflow: If the documentation supports a condition named in this note, do not simply proceed with E85.0: the two are not reported together. The one exception is when the record shows the two conditions are unrelated to each other.

    CompareE34.5, E25.0, D55, Q87.4, E29.1

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

  • Excludes2 — not part of E85.0(3 notes)

    Coding workflow: The conditions named in this note are not included in E85.0. When the record documents both, both may be reported; the note is a boundary, not a prohibition.

    CompareE85.4, G30.0, Q79.6

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

  • Code Also — related condition(2 notes)

    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.

    ReviewM04

    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 E85.0 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).

ReviewE34.5, 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.

Coding context

Guidelines, coding notes, decision aids, relationships (with MS-DRG and CCSR classification), hierarchy, HCC, coverage and the context map: what a coder reaches for after the core. Each section names whether it is official source data, a MedCoder-derived relationship or MedCoder editorial.

Code Overview

Non-neuropathic heredofamilial amyloidosis is a billable ICD-10-CM diagnosis code (E85.0).

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

Decision Points

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

  1. 2 Code Also notes — a second code may apply; the guidelines leave its sequencing to the circumstances of the encounter. See the Code Also notes
  2. 5 Excludes1 entries — codes named there are generally not reported together with this code (Guidelines I.A.12.a). See the Excludes1 notes
  3. 3 Excludes2 entries — 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

  • CC as a secondary diagnosis (FY2027). Can raise the stay's MS-DRG severity tier.

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-derived relationships — computed from published CMS and AHRQ datasets

Other codes that name E85.0 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 8 Excludes1 notes across 4 chapters: E27.1 — Primary adrenocortical insufficiency (via E85.-), K65 — Peritonitis, M60 — Myositis (via E85.-), M60-M63 — Disorders of muscles (M60-M63) (via E85.-), M61 — Calcification and ossification of muscle (via E85.-), M62 — Other disorders of muscle (via E85.-), M63 — Disorders of muscle in diseases classified elsewhere (via E85.-), P59 — Neonatal jaundice from other and unspecified causes (via E85.-).

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

Referenced by 6 Excludes2 notes across 3 chapters: G11 — Hereditary ataxia (via E85.-), G71 — Primary disorders of muscles (via E85.-), K76 — Other diseases of liver (via E85.-), N07 — Hereditary nephropathy, not elsewhere classified (via E85.-), N25.0 — Renal osteodystrophy (via E85.-), N25.81 — Secondary hyperparathyroidism of renal origin (via E85.-).

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

Referenced by 15 Code First instructions across 9 chapters: D77 — Other disorders of blood and blood-forming organs in diseases classified elsewhere (via E85.-), G32.8 — Other specified degenerative disorders of nervous system in diseases classified elsewhere (via E85.-), G63 — Polyneuropathy in diseases classified elsewhere (via E85.-), G99.0 — Autonomic neuropathy in diseases classified elsewhere (via E85.-), G99.8 — Other specified disorders of nervous system in diseases classified elsewhere (via E85.-), H42 — Glaucoma in diseases classified elsewhere (via E85.-), I43 — Cardiomyopathy in diseases classified elsewhere (via E85.-), I68.0 — Cerebral amyloid angiopathy (via E85.-), I79.8 — Other disorders of arteries, arterioles and capillaries in diseases classified elsewhere (via E85.-), J99 — Respiratory disorders in diseases classified elsewhere (via E85.-), K77 — Liver disorders in diseases classified elsewhere (via E85.-), L99 — Other disorders of skin and subcutaneous tissue in diseases classified elsewhere (via E85.-), M14.8 — Arthropathies in other specified diseases classified elsewhere (via E85.-), N08 — Glomerular disorders in diseases classified elsewhere (via E85.-), N29 — Other disorders of kidney and ureter in diseases classified elsewhere (via E85.-).

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.

MS-DRG Grouper Relationships (FY2027)

Potential MS-DRG participation — not a DRG assignment.

FY2027 MS-DRG: CC — Complication or Comorbidity. Reported as a secondary diagnosis, this code raises the stay's MS-DRG severity tier — except when the principal diagnosis is one of 10 clinically related codes on its CMS exclusion list.

Named in the grouper logic of 3 MS-DRGs: DRG 545 (MDC 08), DRG 546 (MDC 08), DRG 547 (MDC 08).

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 clinical process (MS-DRG)

Acts as CC — raises the severity of other admissions. CMS groups these diagnoses into one MS-DRG principal-diagnosis exclusion process — a CC/MCC on this list never raises severity when the principal diagnosis is also on it.

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, E85.9 — Amyloidosis, unspecified, E88.810 — Metabolic syndrome

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.

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, E83.01 — Wilson's disease, E83.09 — Other disorders of copper metabolism, E83.31 — Familial hypophosphatemia, 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, E85.9 — Amyloidosis, unspecified, E88.01 — Alpha-1-antitrypsin deficiency, E88.89 — Other specified metabolic disorders, +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).

E83.59 — Other disorders of calcium metabolism, E83.81 — Hungry bone syndrome, E83.820 — Generalized arterial calcification of infancy with unspecified genetic causality, E83.821 — ENPP1 deficiency causing generalized arterial calcification of infancy, E83.822 — ENPP1 deficiency causing autosomal recessive hypophosphatemic rickets type 2, E83.823 — ABCC6 deficiency causing generalized arterial calcification of infancy, E83.824 — ABCC6 deficiency causing pseudoxanthoma elasticum, E83.825 — CD73 deficiency causing arterial calcification, E83.89 — Other disorders of mineral metabolism, E83.9 — Disorder of mineral metabolism, unspecified, E85.1 — Neuropathic heredofamilial amyloidosis, E85.2 — Heredofamilial amyloidosis, unspecified, E85.3 — Secondary systemic amyloidosis, E85.4 — Organ-limited amyloidosis, E85.8 — Other amyloidosis, E85.81 — Light chain (AL) amyloidosis, E85.82 — Wild-type transthyretin-related (ATTR) amyloidosis, E85.89 — Other amyloidosis, E85.9 — Amyloidosis, unspecified, E88.01 — Alpha-1-antitrypsin deficiency, +258 more

Same Index main term, other category

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

E05.90 — Thyrotoxicosis, unspecified without thyrotoxic crisis or storm (in, thyrotoxicosis), E05.91 — Thyrotoxicosis, unspecified with thyrotoxic crisis or storm (in, thyrotoxicosis, with thyroid storm), E20.9 — Hypoparathyroidism, unspecified (in, hypoparathyroidism), E21.3 — Hyperparathyroidism, unspecified (in, hyperparathyroidism NEC), E23.0 — Hypopituitarism (in, hypopituitarism), E24.9 — Cushing's syndrome, unspecified (in, Cushing's syndrome), E27.1 — Primary adrenocortical insufficiency (in, Addison's disease), E34.9 — Endocrine disorder, unspecified (endocrine NEC), E74.00 — Glycogen storage disease, unspecified (in, glycogen storage disease), E75.6 — Lipid storage disorder, unspecified (in, lipid storage disease), E88.9 — Metabolic disorder, unspecified (in, metabolic disease NEC), G71.02 — Facioscapulohumeral muscular dystrophy (scapulohumeral), G71.035 — Limb girdle muscular dystrophy due to anoctamin-5 dysfunction (Miyoshi, type 3), G71.09 — Other specified muscular dystrophies (ocular), G71.11 — Myotonic muscular dystrophy (proximal myotonic), G71.20 — Congenital myopathy, unspecified (congenital), G71.21 — Nemaline myopathy (rod), G71.220 — X-linked myotubular myopathy (myotubular), G71.228 — Other centronuclear myopathy (centronuclear), G71.29 — Other congenital myopathy (central core), +102 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.

Gamma-glutamyl Transferase (GGT) Test, Lipid Panel, Partial Thromboplastin Time (PTT) Test, Prothrombin Time Test and INR (PT/INR)

Contextual Map

Every relationship of E85.0 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 E85.0 with these 24 related codes in Claim Check

Hierarchy

Excludes2

  • E85.4 — Organ-limited amyloidosis[Excludes2]: “Transthyretin-related (ATTR) familial amyloid cardiomyopathy (E85.4)”— CMS ICD-10-CM tabular instructional notes · icd10cm-fy2027

Code Also

Index manifestation

Referenced by Excludes1 notes

Referenced by Excludes2 notes

Referenced by Code First instructions (15)

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 08 — Diseases and Disorders of the Musculoskeletal System and Connective Tissue[MDC crossing]: “Diseases and Disorders of the Musculoskeletal System and Connective Tissue — 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. 18,439 same-MDC procedures group here; browse them on the MDC page.”— CMS MS-DRG Definitions Manual · FY2027

Index entries

  • Amyloidosis (generalized) (primary), non-neuropathic heredofamilial[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2027
  • Myopathy, in (due to), amyloidosis[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2027
  • Nephropathy, amyloid, hereditary[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2027
  • Peritonitis (adhesive) (bacterial) (fibrinous) (hemorrhagic) (idiopathic) (localized) (perforative) (primary) (with adhesions) (with effusion), paroxysmal, familial[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2027
  • Peritonitis (adhesive) (bacterial) (fibrinous) (hemorrhagic) (idiopathic) (localized) (perforative) (primary) (with adhesions) (with effusion), paroxysmal, familial, benign[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2027
  • Peritonitis (adhesive) (bacterial) (fibrinous) (hemorrhagic) (idiopathic) (localized) (perforative) (primary) (with adhesions) (with effusion), periodic, familial[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2027
  • Polyserositis, periodic, familial[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2027

Nearest codes (10)

Change history

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

Reference

Index terms and tables, published questions and FAQ, every source behind this page with its release and checksum, the date-of-service check and the complete change history.

Indexed Clinical Terms (7)

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.

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 FY2027 tabular list, index and tables, effective October 1, 2026 Release, file and checksum · Publisher’s page
Claim edits Official source data
CMS Definitions of Medicare Code Edits — v44.0 (October 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 — v44 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-derived 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 editorial 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.

Current data releases:ICD-10-CM FY2027 · ICD-10-PCS FY2027 · HCPCS October 2026 · MS-DRG v44 · Medicare Code Editor v44.0 · NCCI PTP Q4 2026 · MUE Q4 2026 · NCD code lists 2026-01 · LCD export September 28, 2026 · All releases and sources

Labels on this page: Official source data · MedCoder-derived relationship · MedCoder editorial explanation. How to read the labels · All data sources and release dates · CMS coding rules

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. "E85.0 — Non-neuropathic heredofamilial amyloidosis." ICD-10-CM FY2027. https://medcoder.ai/icd10/code/e85.0-non-neuropathic-heredofamilial-amyloidosis

Change history

  • FY2016 — October 1, 2015
    In the code set at ICD-10-CM adoption
    Non-neuropathic heredofamilial amyloidosis

No changes since FY2016 — additions, deletions, description changes and billable-status changes are tracked through FY2027, 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 E85.0 in its code family, with their registry titles.

View all codes in the E85 family