E85.1 ICD-10-CM Code: Neuropathic heredofamilial amyloidosis
E85.1 maps to CMS-HCC V28 50. A source-labeled RAF reference is available. Confirm the documented diagnosis and applicable coding requirements. MEAT criteria · RAF Calculator · HCC Buddy coding tools
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FY 2026 Apr update / Endocrine, nutritional and metabolic diseases (E00-E89) / Metabolic disorders (E70-E88)
E85.1
Billable / SpecificICD-10-CMOfficial ICD-10-CMCodebook guidanceNeuropathic heredofamilial amyloidosis
Neuropathic heredofamilial amyloidosis is a genetic disorder causing abnormal protein deposits that damage nerves, leading to progressive weakness and loss of sensation, often starting in the feet.
Buddy Insight
Neuropathic heredofamilial amyloidosis, including familial amyloid polyneuropathy (FAP), involves progressive peripheral and autonomic neuropathy caused by inherited mutations (most commonly transthyretin).
CMS-HCC V28
MappedHCC 50
Code-level coefficient reference
CMS-HCC V24
HistoricalHistorical
Not used for CY2026 payment
ACA/HHS
MappedHCC 029
Code-level coefficient reference
ESRD/PACE
MappedHCC 23
Code-level coefficient reference
RXHCC
N/A—
Not mapped
Code Book Path
Inclusion Terms
Official- Amyloid polyneuropathy (Portuguese)
- Transthyretin-related (ATTR) familial amyloid polyneuropathy
Excludes 2
Official- Ehlers-Danlos syndromes (Q79.6-)Inherited from E70-E88, E85
- Alzheimer's disease (G30.0-)Inherited from E70-E88, E85
Related Codes
Includes
OfficialNo Includes notes are included in this display for E85.1. Check the code and parent instructions in the Code Book.
Excludes 1
Official- transitory endocrine and metabolic disorders specific to newborn (P70-P74)Inherited from E00-E89, E70-E88
- androgen insensitivity syndrome (E34.5-)Inherited from E00-E89, E70-E88
- congenital adrenal hyperplasia (E25.0)Inherited from E00-E89, E70-E88
- hemolytic anemias attributable to enzyme disorders (D55.-)Inherited from E00-E89, E70-E88
- Marfan syndrome (Q87.4-)Inherited from E00-E89, E70-E88
- 5-alpha-reductase deficiency (E29.1)Inherited from E00-E89, E70-E88
Code First
OfficialNo Code First sequencing instructions are included in this display for E85.1. Check the code and parent instructions in the Code Book.
Use Additional
OfficialNo Use Additional Code instructions are included in this display for E85.1. Check the code and parent instructions in the Code Book.
Code Also
OfficialNo Code Also instructions are included in this display for E85.1. Check the code and parent instructions in the Code Book.
Buddy Documentation Tip
MEAT Support
Audit Caution
Common Mistakes
Current with CMS: FY2026 ICD-10-CM Apr 1 update (effective Apr 1 – Sep 30, 2026) · CMS-HCC V28, 100% phased in for payment year 2026. FY2027 code set already staged for October 1, 2026. How HCC Buddy stays current →
Is E85.1 an HCC code?
Yes. E85.1 (Neuropathic heredofamilial amyloidosis) maps to HCC 50, Amyloidosis, Porphyria, and Other Specified Metabolic Disorders under the CMS-HCC V28 risk adjustment model, with a source-labeled community non-dual aged reference coefficient of 0.648. Source-labeled code-level coefficients are references, not member totals. Actual contribution depends on complete member context, hierarchy and cleanup rules, interactions, and model year. HCC Buddy's RAF Calculator supports CMS-HCC V28 PY2026 and shows no score unless every required source and calculation check passes. It is billable for payment year 2026.
Coder answer: E85.1 is billable and maps to V28 HCC 50, Amyloidosis, Porphyria, and Other Specified Metabolic Disorders. Open it in the Code Book for the tabular path, RAF, and MEAT checklist.
- Code
- E85.1
- Description
- Neuropathic heredofamilial amyloidosis
- HCC (V28)
- HCC 50 — Amyloidosis, Porphyria, and Other Specified Metabolic Disorders
- RAF reference coefficient
- 0.648
- Billable
- Yes
- Payment year
- 2026
HCC Category Mapping
These are source-labeled model coefficients, not member totals. A category may still be removed by hierarchy or model cleanup rules. Weights are not directly comparable across models: CMS-HCC V28 and V24 use Community, Non-Dual, Aged; ESRD uses the dialysis continuing-enrollee model; RxHCC is the Part D continuing-enrollee, non-low-income, aged weight (a larger scale than CMS-HCC). ACA/HHS has no single weight — it varies by metal level. Actual per-patient RAF contribution depends on member context, hierarchy and cleanup rules, interactions, and the model year used by the payer. V28 is the CMS-HCC model phased in over payment years 2024–2026; V24 remains available for historical review.
Work E85.1 in the Code Book — tabular path, V28 RAF reference, and MEAT checklist →
MEAT review for E85.1
For E85.1, confirm that the documentation supports the diagnosis and meets the applicable coding, encounter, program and payer requirements. MEAT (Monitor, Evaluate, Assess, or Treat) is a review mnemonic, not a universal CMS coding rule.
- MMonitor: signs, symptoms, disease progression, or lab trending documented in the note
- EEvaluate: test results, medication response, or physical findings reviewed by the provider
- AAssess: explicit mention in the assessment or plan with acknowledgment of status
- TTreat: medication, referral, procedure, therapy, or counseling tied to the diagnosis
Coder workflow notes
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What This Code Means
E85.1 is the ICD-10-CM diagnosis code for neuropathic heredofamilial amyloidosis. Neuropathic heredofamilial amyloidosis is a genetic disorder causing abnormal protein deposits that damage nerves, leading to progressive weakness and loss of sensation, often starting in the feet. E85.1 sits in the ICD-10-CM chapter for endocrine, nutritional and metabolic diseases (e00-e89), within the section covering metabolic disorders (e70-e88).
Under the CMS-HCC V28 risk adjustment model, E85.1 maps to Amyloidosis, Porphyria, and Other Specified Metabolic Disorders (HCC 50) with a source-labeled community, non-dual, aged reference coefficient of 0.648. No V24 mapping is shown for E85.1; use the applicable model and payment year when reviewing the V28 mapping. For CY2026 non-PACE Medicare Advantage, CMS uses 100% of the 2024 CMS-HCC model (V28). PACE uses a separate model blend. Source-labeled code-level coefficients are references, not member totals. Actual contribution depends on complete member context, hierarchy and cleanup rules, interactions, and model year. HCC Buddy's RAF Calculator supports CMS-HCC V28 PY2026 and shows no score unless every required source and calculation check passes.
Confirm the presence of neuropathy symptoms in documentation; this distinguishes it from non-neuropathic amyloidosis. For E85.1, confirm that the documentation supports the diagnosis and meets the applicable coding, encounter, program and payer requirements. MEAT (Monitor, Evaluate, Assess, or Treat) is a review mnemonic, not a universal CMS coding rule. When documentation is ambiguous, coders should issue a provider query rather than assume the highest-specificity variant.
HCC Buddy maintains structured V28 and V24 mapping, source-labeled coefficient references, and MEAT documentation criteria for E85.1 sourced directly from the CMS-HCC risk adjustment model files and the CMS ICD-10-CM code set.
Coding Tips
- •Confirm the presence of neuropathy symptoms in documentation; this distinguishes it from non-neuropathic amyloidosis
- •Code any associated organ involvement separately if significant (e.g., cardiac amyloidosis)
Clinical Significance
Neuropathic heredofamilial amyloidosis, including familial amyloid polyneuropathy (FAP), involves progressive peripheral and autonomic neuropathy caused by inherited mutations (most commonly transthyretin). Disease-modifying therapies like tafamidis and gene-silencing agents make accurate diagnosis and coding essential for treatment access and risk adjustment.
Documentation Requirements
- ✓Confirmed amyloidosis diagnosis with tissue biopsy or genetic confirmation
- ✓Hereditary/familial etiology established (TTR mutation, other amyloidogenic mutation)
- ✓Documentation of neuropathic manifestations (peripheral neuropathy, autonomic neuropathy)
- ✓Nerve conduction studies or other neurologic assessment
- ✓Current disease-modifying therapy (tafamidis, patisiran, inotersen)
- ✓Assessment of non-neurologic organ involvement (cardiac, renal, GI)
Commonly Confused Codes
- •E85.0: Non-neuropathic heredofamilial amyloidosis: hereditary form WITHOUT neuropathy as primary feature
- •E85.82: Wild-type transthyretin-related (ATTR) amyloidosis: age-related, NOT hereditary
- •E85.81: Light chain (AL) amyloidosis: acquired form from plasma cell disorder
- •G63: Polyneuropathy in diseases classified elsewhere: may be used additionally but does not replace the amyloidosis code