E71.111 ICD-10-CM Code: 3-methylglutaconic aciduria
E71.111 is not a CMS-HCC payment code. 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)
E71.111
Billable / SpecificICD-10-CMOfficial ICD-10-CMCodebook guidance3-methylglutaconic aciduria
A rare genetic metabolic disorder where the body accumulates 3-methylglutaconic acid due to an enzyme deficiency, which can affect muscle and neurological function.
Buddy Insight
3-methylglutaconic aciduria is a rare inborn error of leucine metabolism that can present with neurological dysfunction, muscle weakness, and lactic acidosis.
CMS-HCC V28
N/A—
Not mapped
CMS-HCC V24
HistoricalHistorical
Not used for CY2026 payment
ACA/HHS
Context neededHCC 028
Coefficient needs member context
ESRD/PACE
Context neededHCC 23
Coefficient needs member context
RXHCC
MappedHCC 43
Code-level coefficient reference
Code Book Path
Inclusion Terms
OfficialNo inclusion terms are included in this display for E71.111. Check the code and parent instructions in the Code Book.
Excludes 2
Official- Ehlers-Danlos syndromes (Q79.6-)Inherited from E70-E88
Related Codes
Includes
OfficialNo Includes notes are included in this display for E71.111. 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 E71.111. Check the code and parent instructions in the Code Book.
Use Additional
OfficialNo Use Additional Code instructions are included in this display for E71.111. Check the code and parent instructions in the Code Book.
Code Also
OfficialNo Code Also instructions are included in this display for E71.111. 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 E71.111 an HCC code?
E71.111 has no mapping under the current CMS-HCC V28 community payment model. E71.111 has a separate mapping under the CMS-HCC ESRD model (HCC 23 (Other Significant Endocrine and Metabolic Disorders)) and the Part D RxHCC model (HCC 43 (Pituitary, Adrenal Gland, and Other Endocrine and Metabolic Disorders)); the applicable result needs member context. E71.111 also appears in the HHS-HCC commercial risk model (HCC 028 (HHS-HCC 028 child, RAF varies by metal level)), where the result depends on member context and is not a Medicare Advantage payment mapping.
- Code
- E71.111
- Description
- 3-methylglutaconic aciduria
- HCC (V28)
- No CMS-HCC V28 mapping
- RAF reference coefficient
- —
- 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 E71.111 in the Code Book — tabular path, V28 RAF reference, and MEAT checklist →
MEAT review for E71.111
For E71.111, 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
E71.111 is the ICD-10-CM diagnosis code for 3-methylglutaconic aciduria. A rare genetic metabolic disorder where the body accumulates 3-methylglutaconic acid due to an enzyme deficiency, which can affect muscle and neurological function. E71.111 sits in the ICD-10-CM chapter for endocrine, nutritional and metabolic diseases (e00-e89), within the section covering metabolic disorders (e70-e88).
E71.111 has no mapping under the current CMS-HCC V28 community payment model. E71.111 has a separate mapping under the CMS-HCC ESRD model (HCC 23 (Other Significant Endocrine and Metabolic Disorders)) and the Part D RxHCC model (HCC 43 (Pituitary, Adrenal Gland, and Other Endocrine and Metabolic Disorders)); the applicable result needs member context. E71.111 also appears in the HHS-HCC commercial risk model (HCC 028 (HHS-HCC 028 child, RAF varies by metal level)), where the result depends on member context and is not a Medicare Advantage payment mapping. Do not assign V28 risk adjustment value from this page; verify the applicable model and payment year before using this code for risk adjustment.
This is a specific organic aciduria code; verify it is not being confused with other branched-chain disorders.
HCC Buddy maintains structured V28 and V24 mapping, source-labeled coefficient references, and MEAT documentation criteria for E71.111 sourced directly from the CMS-HCC risk adjustment model files and the CMS ICD-10-CM code set.
Coding Tips
- •This is a specific organic aciduria code; verify it is not being confused with other branched-chain disorders
- •Document any associated symptoms or complications for complete clinical picture
Clinical Significance
3-methylglutaconic aciduria is a rare inborn error of leucine metabolism that can present with neurological dysfunction, muscle weakness, and lactic acidosis. It encompasses several subtypes with varying severity, some associated with cardiomyopathy and optic atrophy. Early diagnosis through urine organic acid analysis is critical for management and genetic counseling.
Documentation Requirements
- ✓Document the specific subtype if known (types I-V), results of urine organic acid testing showing elevated 3-methylglutaconic acid, associated neurological findings, cardiac involvement, and any genetic testing results.
- ✓Record current symptom severity and management plan.