2026 ICD-10-CM Diagnosis Code E85.1Neuropathic heredofamilial amyloidosis

ICD-10-CM Codes›E00–E89›E70-E88›E85

ICD-10-CM E85.1
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

E85.1 is a billable ICD-10-CM diagnosis code for neuropathic heredofamilial amyloidosis. It is valid on HIPAA claims for fiscal year 2026 (October 1, 2025 through September 30, 2026) and groups to MS-DRG 545 through 547. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Other specified and unspecified nutritional and metabolic disorders.

Code Identity

ICD-10-CM Code
E85.1
Billable Status
Yes — Valid for Submission
Code Describes
Neuropathic heredofamilial amyloidosis
Short Description
Neuropathic heredofamilial amyloidosis
Same as the full description in the CMS dataset.
Parent Code
E85
Amyloidosis

Code Classification

ChapterE00–E89Endocrine, nutritional and metabolic diseases
SectionE70-E88Metabolic disorders
CategoryE85Amyloidosis
This CodeE85.1Neuropathic heredofamilial amyloidosis

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Amyloid polyneuropathy type I
  • Cerebral amyloid angiopathy
  • 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

Tabular List NotesGuidance

Coding notes and annotation back-references applicable to this code.

Inclusion Terms

  • Amyloid polyneuropathy (Portuguese)
  • Transthyretin-related (ATTR) familial amyloid polyneuropathy

Index to Diseases and InjuriesGuidance

Alphabetical index entries that point to this code.

External Cause of Injuries IndexGuidance

References for this 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

Clinical ClassificationClinical

AHRQ’s CCSR groups this code into broader clinical categories.

CCSR END016
Other specified and unspecified nutritional and metabolic disorders
Default principal diagnosis: inpatient Yes · outpatient Yes

Clinical InformationClinical

  • 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.

Patient EducationClinical

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.

Read the full article at MedlinePlus

Courtesy of MedlinePlus, a service of the U.S. National Library of Medicine.

Convert E85.1 to ICD-9-CMHistory

The closest ICD-9-CM equivalents under the General Equivalence Mappings.

ICD-9-CM
277.39 Amyloidosis NEC
Approximate The match is approximate rather than exact.

Code HistoryHistory

FY 2016AddedAdded to the ICD-10-CM code setEffective October 1, 2015, the first year of ICD-10-CM.
FY 2017–2025No changes
FY 2026CurrentCurrent code set, no changesEffective October 1, 2025 through September 30, 2026.

Questions About E85.1Overview

Is E85.1 (Amyloidosis) a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report neuropathic heredofamilial amyloidosis on HIPAA-covered claims from October 1, 2025 through September 30, 2026.

What MS-DRG does E85.1 group to?

When neuropathic heredofamilial amyloidosis is the principal diagnosis on an inpatient stay, it groups to MS-DRG 545, 546, 547, with relative weights from 0.8362 to 2.4817 depending on complications. Higher weights mean higher Medicare reimbursement.

What is the ICD-9 equivalent of E85.1?

Under the General Equivalence Mappings, neuropathic heredofamilial amyloidosis converts to ICD-9-CM 277.39 (amyloidosis NEC). The mapping is approximate, so confirm the match fits the documentation.

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.