E79.81
BillableAicardi-Goutières syndrome
HCC Category Mapping
V24HCC 23 — Other Significant Endocrine and Metabolic Disorders
0.230ESRDHCC 23 — Other Significant Endocrine and Metabolic Disorders
0.000RxHCCHCC 43 — Other Significant Endocrine and Metabolic Disorders
0.000What This Code Means
A rare inherited autoimmune-like condition affecting the nervous system, causing progressive brain and nerve damage.
Coding Tips
- •This is a complex genetic disorder; ensure complete documentation of all manifestations
- •Code associated neurological symptoms separately as they develop
Clinical Significance
Aicardi-Goutieres syndrome is a severe genetic autoinflammatory disorder that mimics congenital viral infection, causing progressive encephalopathy, intracranial calcifications, white matter disease, and elevated interferon-alpha in cerebrospinal fluid. It is an interferonopathy that causes significant disability and requires complex multidisciplinary care.
Documentation Requirements
- ✓Provider diagnosis of Aicardi-Goutieres syndrome
- ✓Genetic testing confirming mutation in one of the associated genes (TREX1, RNASEH2A, RNASEH2B, RNASEH2C, SAMHD1, ADAR1, IFIH1)
- ✓Neuroimaging findings: intracranial calcifications, white matter abnormalities, cerebral atrophy
- ✓Cerebrospinal fluid analysis: elevated interferon-alpha, lymphocytosis
- ✓Current neurological status: encephalopathy severity, seizures, motor function
- ✓Chilblain-like skin lesions if present
Commonly Confused Codes
P35.0-P35.9 — Congenital viral diseases: Aicardi-Goutieres mimics congenital TORCH infections but is geneticE79.89 — Other disorders of purine and pyrimidine metabolism: broader category within purine/pyrimidine disordersG31.89 — Other degenerative diseases of nervous system: Aicardi-Goutieres is metabolic/autoinflammatoryG80.9 — Cerebral palsy, unspecified: early motor impairment may be misdiagnosed as cerebral palsy