2026 ICD-10-CM Diagnosis Code E79.81Aicardi-Goutieres syndrome

ICD-10-CM Codes›E00–E89›E70-E88›E79

ICD-10-CM E79.81
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

E79.81 is a billable ICD-10-CM diagnosis code for Aicardi-Goutieres syndrome. It is valid on HIPAA claims for fiscal year 2026 (October 1, 2025 through September 30, 2026). 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
E79.81
Billable Status
Yes — Valid for Submission
Code Describes
Aicardi-Goutieres syndrome
Short Description
Aicardi-Goutieres syndrome
Same as the full description in the CMS dataset.
Parent Code
E79.8
Other disorders of purine and pyrimidine metabolism

Code Classification

ChapterE00–E89Endocrine, nutritional and metabolic diseases
SectionE70-E88Metabolic disorders
CategoryE79Disorders of purine and pyrimidine metabolism
This CodeE79.81Aicardi-Goutieres syndrome

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.

    • Syndrome
      • Aicardi-Goutières

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

Code History & ChangesHistory

Replacement E79.81 replaces the following previously assigned code(s):

  • E79.8 - Other disorders of purine and pyrimidine metabolism
FY 2024AddedAdded to the ICD-10-CM code setEffective October 1, 2023.
FY 2025No changes
FY 2026CurrentCurrent code set, no changesEffective October 1, 2025 through September 30, 2026.

Questions About E79.81Overview

Is E79.81 (Other disorders of purine and pyrimidine metabolism) a billable code?

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

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.