E83.821 ICD-10-CM Code: ENPP1 deficiency causing generalized arterial calcification of infancy
HCC Buddy Code Card
Digital ICD-10 code-book layout with official code detail, always-visible risk models, Code Trumping, and Buddy coding guidance.
FY 2026 Apr update / Endocrine, nutritional and metabolic diseases (E00-E89) / Metabolic disorders (E70-E88)
E83.821
Billable / SpecificICD-10-CMOfficial ICD-10-CMCodebook guidanceENPP1 deficiency causing generalized arterial calcification of infancy
A rare genetic disorder caused by ENPP1 gene deficiency that leads to abnormal calcium and phosphate deposits in arteries, typically appearing in infancy.
CMS-HCC V28
N/A—
Not mapped
CMS-HCC V24
N/A—
Not mapped
ACA/HHS
MappedHCC 28
Varies by metal level
ESRD/PACE
MappedHCC 23
RAF 0.036
RXHCC
MappedHCC 43
RAF 0.063
Code Book Path
Inclusion Terms
OfficialICD-10-CM does not list inclusion terms for E83.821 in this effective period.
Excludes 2
OfficialICD-10-CM does not list Excludes 2 notes for E83.821 in this effective period.
Related Child Codes
Includes
OfficialICD-10-CM does not list Includes notes for E83.821 in this effective period.
Excludes 1
OfficialICD-10-CM does not list Excludes 1 notes for E83.821 in this effective period.
Code First
OfficialICD-10-CM does not list Code First sequencing instructions for E83.821 in this effective period.
Use Additional
OfficialICD-10-CM does not list Use Additional Code instructions for E83.821 in this effective period.
Code Also
Official- , if applicable, associated conditions such as:
- heart failure (I50.-)
- other secondary hypertension (I15.8)
Buddy Documentation Tip
Last updated: FY2026 ICD-10-CM Apr update, Apr 1, 2026 through Sep 30, 2026. CMS-HCC V28 is 100% phased in for payment year 2026.
Is E83.821 an HCC code?
E83.821 is not in the CMS-HCC V28 or V24 community payment model, but it does map to Other Significant Endocrine and Metabolic Disorders under the CMS-HCC ESRD model and Other Significant Endocrine and Metabolic Disorders under the Part D RxHCC model.
HCC Category Mapping
Each model's RAF is its CMS base weight for that model's standard population, so 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 segment, interactions, and the model year used by the payer. V28 is the CMS-HCC model phased in over payment years 2024–2026; V24 remains in use during the transition and for historical data.
Work E83.821 in the Code Book — tabular path, V28 RAF, and MEAT checklist →
MEAT Criteria for E83.821
For E83.821to count as a valid HCC diagnosis in a given encounter, the provider's documentation must show MEAT: Monitor, Evaluate, Assess, or Treat. A diagnosis from a prior year does not carry forward automatically, it has to be re-documented and supported each calendar year.
- 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
Only one of M/E/A/T is required to support the code, but the documentation must be specific enough to show that the provider actually addressed E83.821 during that encounter, not just copy-forwarded from a problem list.
Coder workflow notes
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What This Code Means
E83.821 is the ICD-10-CM diagnosis code for enpp1 deficiency causing generalized arterial calcification of infancy. A rare genetic disorder caused by ENPP1 gene deficiency that leads to abnormal calcium and phosphate deposits in arteries, typically appearing in infancy. E83.821 sits in the ICD-10-CM chapter for endocrine, nutritional and metabolic diseases (e00-e89), within the section covering metabolic disorders (e70-e88).
E83.821 is a billable ICD-10-CM code but does not map to a payment HCC under the CMS-HCC V28, V24, ESRD, or RxHCC risk adjustment models. It can be reported on Medicare Advantage encounter data submissions but it does not contribute to a beneficiary's RAF score and therefore does not affect risk-adjusted payments to the plan.
This is a specific genetic disorder requiring confirmation of ENPP1 deficiency through genetic testing documentation.
HCC Buddy maintains structured V28 and V24 mapping, RAF weights, and MEAT documentation criteria for E83.821 sourced directly from the CMS-HCC risk adjustment model files and the CMS ICD-10-CM code set.
Coding Tips
- •This is a specific genetic disorder requiring confirmation of ENPP1 deficiency through genetic testing documentation
- •Code any associated complications such as vascular calcification or cardiovascular manifestations separately