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  6. 2026 ICD-10-CM Code E85.1

2026 ICD-10-CM Diagnosis Code E85.1

Neuropathic heredofamilial amyloidosis

ICD-10-CM Code:
E85.1
ICD-10 Code for:
Neuropathic heredofamilial amyloidosis
Is Billable?
Yes - Valid for Submission
Chronic Condition Indicator: [1]
Chronic
Code Navigator:

E85.1 is a billable diagnosis code used to specify a medical diagnosis of neuropathic heredofamilial amyloidosis. The code is valid during the current fiscal year for the submission of HIPAA-covered transactions from October 01, 2025 through September 30, 2026.

Code Classification

  • Endocrine, nutritional and metabolic diseases
    E00–E89

Approximate Synonyms

The following list of clinical terms are approximate synonyms, alternative descriptions, or common phrases that might be used by patients, healthcare providers, or medical coders to describe the same condition. These synonyms and related diagnosis terms are often used when searching for an ICD-10 code, especially when the exact medical terminology is unclear. Whether you're looking for lay terms, similar diagnosis names, or common language alternatives, this list can help guide you to the correct ICD-10 classification.

  • Amyloid polyneuropathy type I
  • Cerebral amyloid angiopathy
  • Familial amyloid polyneuropathy
  • Familial amyloid polyneuropathy
  • Familial amyloid polyneuropathy with cutaneous amyloidosis
  • Familial amyloid polyneuropathy, Iowa type
  • Familial amyloid polyneuropathy, Jewish type
  • Familial amyloid polyneuropathy, type II
  • Familial amyloid polyneuropathy, type VI
  • Glomerular disease due to familial disease
  • Glomerular disorder due to amyloidosis
  • Glomerular disorder due to neuropathic heredofamilial amyloidosis
  • Hereditary ATTR amyloidosis
  • Hereditary cerebral amyloid angiopathy, Icelandic type
  • Hereditary cerebral hemorrhage with amyloidosis
  • Hereditary cerebrovascular amyloidosis
  • Heredofamilial systemic amyloidosis affecting skin
  • Localized hereditary amyloidosis
  • Neuropathy associated with dysproteinemias
  • Polyneuropathy due to amyloidosis
  • Systemic amyloidosis affecting skin

Clinical Classification

Clinical Classifications group individual ICD-10-CM diagnosis codes into broader, clinically meaningful categories. These categories help simplify complex data by organizing related conditions under common clinical themes.

They are especially useful for data analysis, reporting, and clinical decision-making. Even when diagnosis codes differ, similar conditions can be grouped together based on their clinical relevance. Each category is assigned a unique CCSR code that represents a specific clinical concept, often tied to a body system or medical specialty.

Other specified and unspecified nutritional and metabolic disorders

CCSR Code: END016

Inpatient Default: Y - Yes, default inpatient assignment for principal diagnosis or first-listed diagnosis.

Outpatient Default: Y - Yes, default outpatient assignment for principal diagnosis or first-listed diagnosis.

Clinical Information

  • Amyloid Neuropathies, Familial

    inherited disorders of the peripheral nervous system associated with the deposition of amyloid in nerve tissue. the different clinical types based on symptoms correspond to the presence of a variety of mutations in several different proteins including transthyretin (prealbumin); apolipoprotein a-i; and gelsolin.

  • Cerebral Amyloid Angiopathy

    a heterogeneous group of sporadic or familial disorders characterized by amyloid deposits in the walls of small and medium sized blood vessels of cerebral cortex and meninges. clinical features include multiple, small lobar cerebral hemorrhage; cerebral ischemia (brain ischemia); and cerebral infarction. cerebral amyloid angiopathy is unrelated to generalized amyloidosis. amyloidogenic peptides in this condition are nearly always the same ones found in alzheimer disease. (from kumar: robbins and cotran: pathologic basis of disease, 7th ed., 2005)

  • Cerebral Amyloid Angiopathy, Familial

    a familial disorder marked by amyloid deposits in the walls of small and medium sized blood vessels of cerebral cortex and meninges.

  • Cerebral Amyloid Angiopathy

    a disorder characterized by the deposition of amyloid in the wall of the vessels in the brain.

  • Cerebral Amyloid Angiopathy, APP-Related|HCHWAD|Hereditary Cerebral Hemorrhage with Amyloidosis-Dutch Type

    an autosomal dominant form of cerebral amyloid angiopathy caused by mutation(s) in the app gene, encoding amyloid-beta a4 protein. the deposition of amyloid in cerebral blood vessels wall may lead to degenerative vascular changes that may result in cerebral hemorrhage. mutation(s) in the app gene may also cause autosomal dominant alzheimer disease 1.

Tabular List of Diseases and Injuries

The following annotation back-references are applicable to this diagnosis code. The Tabular List of Diseases and Injuries is a list of ICD-10-CM codes, organized "head to toe" into chapters and sections with coding notes and guidance for inclusions, exclusions, descriptions and more.


Inclusion Terms

Inclusion Terms
These terms are the conditions for which that code is to be used. The terms may be synonyms of the code title, or, in the case of "other specified" codes, the terms are a list of the various conditions assigned to that code. The inclusion terms are not necessarily exhaustive. Additional terms found only in the Alphabetic Index may also be assigned to a code.
  • Amyloid polyneuropathy (Portuguese)
  • Transthyretin-related (ATTR) familial amyloid polyneuropathy

Index to Diseases and Injuries References

The following annotation back-references for this diagnosis code are found in the injuries and diseases index. The Index to Diseases and Injuries is an alphabetical listing of medical terms, with each term mapped to one or more ICD-10-CM code(s).

Index of External Cause of Injuries

References found for this diagnosis code in the External Cause of Injuries Index:

    • Amyloidosis(generalized) (primary)
      • neuropathic heredofamilial
    • Amyloidosis(generalized) (primary)
      • Portuguese
    • Polyneuropathy(peripheral)
      • amyloid (Portuguese)
    • Polyneuropathy(peripheral)
      • amyloid (Portuguese)
        • transthyretin-related (ATTR) familial
    • Polyneuropathy(peripheral)
      • in (due to)
        • amyloidosis, familial (Portuguese)
    • Polyneuropathy(peripheral)
      • in (due to)
        • transthyretin-related (ATTR) familial amyloid

Convert E85.1 to ICD-9-CM

Below are the ICD-9 codes that most closely match this ICD-10 code, based on the General Equivalence Mappings (GEMs). This ICD-10 to ICD-9 crosswalk tool is helpful for coders who need to reference legacy diagnosis codes for audits, historical claims, or approximate code comparisons.

Amyloidosis NEC

ICD-9-CM: 277.39

Approximate Flag - The approximate mapping means this ICD-10 code does not have an exact ICD-9 equivalent. The matched code is the closest available option, but it may not fully capture the original diagnosis or clinical intent.

View Conversion

Patient Education


Amyloidosis

Amyloidosis occurs when abnormal proteins called amyloids build up and form deposits. The deposits can collect in organs such as the kidney and heart. This can cause the organs to become stiff and unable to work the way they should.

There are three main types of amyloidosis:

  • Primary - with no known cause
  • Secondary - caused by another disease, including some types of cancer
  • Familial - passed down through genes

Symptoms can vary, depending upon which organs are affected. Treatment depends on the type of amyloidosis you have. The goal is to help with symptoms and limit the production of proteins. If another disease is the cause, it needs to be treated.


[Learn More in MedlinePlus]

Code History

  • FY 2026 - No Change, effective from 10/1/2025 through 9/30/2026
  • FY 2025 - No Change, effective from 10/1/2024 through 9/30/2025
  • FY 2024 - No Change, effective from 10/1/2023 through 9/30/2024
  • FY 2023 - No Change, effective from 10/1/2022 through 9/30/2023
  • FY 2022 - No Change, effective from 10/1/2021 through 9/30/2022
  • FY 2021 - No Change, effective from 10/1/2020 through 9/30/2021
  • FY 2020 - No Change, effective from 10/1/2019 through 9/30/2020
  • FY 2019 - No Change, effective from 10/1/2018 through 9/30/2019
  • FY 2018 - No Change, effective from 10/1/2017 through 9/30/2018
  • FY 2017 - No Change, effective from 10/1/2016 through 9/30/2017
  • FY 2016 - New Code, effective from 10/1/2015 through 9/30/2016. This was the first year ICD-10-CM was implemented into the HIPAA code set.

Footnotes

[1] Chronic - a chronic condition code indicates a condition lasting 12 months or longer and its effect on the patient based on one or both of the following criteria:

  • The condition results in the need for ongoing intervention with medical products,treatment, services, and special equipment
  • The condition places limitations on self-care, independent living, and social interactions.