2026 ICD-10-CM Diagnosis Code E83.821ENPP1 deficiency causing generalized arterial calcification of infancy

ICD-10-CM Codes›E00–E89›E70-E88›E83

ICD-10-CM E83.821
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

E83.821 is a billable ICD-10-CM diagnosis code for ENPP1 deficiency causing generalized arterial calcification of infancy. It is valid on HIPAA claims for fiscal year 2026 (October 1, 2025 through September 30, 2026) and groups to MS-DRG 299 through 301.

Code Identity

ICD-10-CM Code
E83.821
Billable Status
Yes — Valid for Submission
Code Describes
ENPP1 deficiency causing generalized arterial calcification of infancy
Short Description
ENPP1 deficiency causing gen arterial calcifcn of infancy
Parent Code
E83.82
Disorders of pyrophosphate metabolism

Code Classification

ChapterE00–E89Endocrine, nutritional and metabolic diseases
SectionE70-E88Metabolic disorders
CategoryE83Disorders of mineral metabolism
This CodeE83.821ENPP1 deficiency causing generalized arterial calcification of infancy

Tabular List NotesGuidance

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

Code Also

  • , if applicable, associated conditions such as:
  • heart failure I50
  • other secondary hypertension I15.8

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.

    • Calcification
      • arterial
        • generalized, of infancy
          • with
            • ENPP1 deficiency
    • Deficiency, deficient
      • ENPP1
        • causing
          • generalized arterial calcification of infancy

Code History & ChangesHistory

New Code E83.821 was added to the ICD-10-CM code set for FY 2026, effective October 1, 2025.

Replacement E83.821 replaces the following previously assigned code(s):

  • E83.89 - Other disorders of mineral metabolism
FY 2026AddedAdded to the ICD-10-CM code setEffective October 1, 2025.
FY 2026CurrentRevised in the current code setEffective October 1, 2025 through September 30, 2026.

Questions About E83.821Overview

Is E83.821 (Disorders of pyrophosphate metabolism) a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report ENPP1 deficiency causing generalized arterial calcification of infancy on HIPAA-covered claims from October 1, 2025 through September 30, 2026.

What MS-DRG does E83.821 group to?

When ENPP1 deficiency causing generalized arterial calcification of infancy is the principal diagnosis on an inpatient stay, it groups to MS-DRG 299, 300, 301, with relative weights from 0.7197 to 1.6327 depending on complications. Higher weights mean higher Medicare reimbursement.