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E74.02 ICD-10-CM Code: Pompe disease

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FY 2026 Apr update / Endocrine, nutritional and metabolic diseases (E00-E89) / Metabolic disorders (E70-E88)

E74.02

Billable / SpecificICD-10-CMOfficial ICD-10-CMCodebook guidance

Pompe disease

A rare inherited disorder where the body cannot break down glycogen due to acid maltase enzyme deficiency, causing muscle weakness and heart problems.

Buddy the Bee presenting code insight

Buddy Insight

Pompe disease (Glycogen Storage Disease Type II) is caused by acid alpha-glucosidase (acid maltase) deficiency, leading to progressive glycogen accumulation in muscles including the heart and diaphragm.

CMS-HCC V28

HCC 49

RAF 0.226

CMS-HCC V24

HCC 23

RAF 0.230

ACA/HHS

0

0

RAF 0

ESRD/PACE

HCC 23

RAF 0.0

RXHCC

HCC 41

RAF 0.0

Code Trumping

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Code Book Path

Official
E74Other disorders of carbohydrate metabolism
E74.0Glycogen storage disease
E74.02Pompe disease

Inclusion Terms

Official
  • Cardiac glycogenosis
  • Type II glycogen storage disease

Excludes 2

Official

ICD-10-CM does not list Excludes 2 notes for E74.02 in this effective period.

Related Child Codes

Official
E74.00Glycogen storage disease, unspecified
E74.01von Gierke disease
E74.03Cori disease
E74.04McArdle disease
E74.05Lysosome-associated membrane protein 2 [LAMP2] deficiency

Includes

Official

ICD-10-CM does not list Includes notes for E74.02 in this effective period.

Excludes 1

Official

ICD-10-CM does not list Excludes 1 notes for E74.02 in this effective period.

Code First

Official

ICD-10-CM does not list Code First sequencing instructions for E74.02 in this effective period.

Use Additional

Official

ICD-10-CM does not list Use Additional Code instructions for E74.02 in this effective period.

Code Also

Official

ICD-10-CM does not list Code Also instructions for E74.02 in this effective period.

Buddy Documentation Tip

HCC Buddy guidance
Confirmed diagnosis of Pompe disease or GSD Type II
Acid alpha-glucosidase enzyme assay results or GAA gene mutation analysis
Form specification: infantile-onset, late-onset, or childhood-onset
Cardiac evaluation including echocardiogram findings if infantile form

MEAT Support

HCC Buddy guidance
Confirmed diagnosis of Pompe disease or GSD Type II
Acid alpha-glucosidase enzyme assay results or GAA gene mutation analysis
Form specification: infantile-onset, late-onset, or childhood-onset
Cardiac evaluation including echocardiogram findings if infantile form

Audit Caution

HCC Buddy guidance
Coding only the cardiomyopathy or myopathy without identifying the underlying Pompe disease
Failing to distinguish infantile-onset from late-onset Pompe, which have different clinical trajectories
Not coding respiratory failure or ventilator dependence as additional diagnoses when present
Confusing Pompe disease with other causes of hypertrophic cardiomyopathy in infants

Common Mistakes

HCC Buddy guidance
E74.00 — Glycogen storage disease, unspecified: do not use when Pompe disease is documented
E74.05 — LAMP2 deficiency (Danon disease): also causes cardiomyopathy with glycogen accumulation but is a different enzyme
E74.04 — McArdle disease: muscle-only GSD but involves myophosphorylase, not acid maltase
I42.8 — Other cardiomyopathies: cardiomyopathy is a manifestation of Pompe, code the underlying cause

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 E74.02 an HCC code?

Yes. E74.02 maps to Lysosomal Storage Disorders under the CMS-HCC V28 risk adjustment model (and Other Significant Endocrine and Metabolic Disorders under V24).

HCC Category Mapping

V28HCC 49, Lysosomal Storage Disorders
0.226
V24HCC 23, Other Significant Endocrine and Metabolic Disorders
0.230
ESRDHCC 23, Other Significant Endocrine and Metabolic Disorders
0.000
RxHCCHCC 41, Lysosomal Storage Disorders
0.000

RAF weights shown are the community, non-dual, aged base weights from the CMS risk adjustment model file. 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.

MEAT Criteria for E74.02

For E74.02to 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 E74.02 during that encounter, not just copy-forwarded from a problem list.

What This Code Means

E74.02 is the ICD-10-CM diagnosis code for pompe disease. A rare inherited disorder where the body cannot break down glycogen due to acid maltase enzyme deficiency, causing muscle weakness and heart problems. E74.02 sits in the ICD-10-CM chapter for endocrine, nutritional and metabolic diseases (e00-e89), within the section covering metabolic disorders (e70-e88).

Under the CMS-HCC V28 risk adjustment model, E74.02 maps to Lysosomal Storage Disorders (HCC 49) with a community, non-dual, aged base RAF weight of 0.226. Under the older CMS-HCC V24 model, E74.02 maps to Other Significant Endocrine and Metabolic Disorders (HCC 23) with a community, non-dual, aged base RAF weight of 0.230. V28 is the CMS-HCC risk adjustment model that reached 100% phase-in for payment year 2026, replacing V24 which was used during the PY2024–PY2025 transition.

Document the age of onset (infantile, juvenile, or adult form) as this affects severity and prognosis. Because E74.02 maps to a payment HCC, the provider's documentation must satisfy MEAT criteria (Monitor, Evaluate, Assess, or Treat) for the encounter to count toward the patient's Medicare Advantage risk adjustment score. When documentation is ambiguous, coders should issue a provider query rather than assume the highest-specificity variant.

HCC Buddy maintains structured V28 and V24 mapping, RAF weights, and MEAT documentation criteria for E74.02 sourced directly from the CMS-HCC risk adjustment model files and the CMS ICD-10-CM code set.

Coding Tips

  • •Document the age of onset (infantile, juvenile, or adult form) as this affects severity and prognosis
  • •Note any cardiac involvement or respiratory muscle weakness, as these are key features of Pompe disease

Clinical Significance

Pompe disease (Glycogen Storage Disease Type II) is caused by acid alpha-glucosidase (acid maltase) deficiency, leading to progressive glycogen accumulation in muscles including the heart and diaphragm. The infantile form causes fatal cardiomyopathy, while late-onset forms cause progressive limb-girdle weakness and respiratory failure. Enzyme replacement therapy (Lumizyme/Myozyme) has transformed outcomes but requires lifelong infusions.

Documentation Requirements

  • ✓Confirmed diagnosis of Pompe disease or GSD Type II
  • ✓Acid alpha-glucosidase enzyme assay results or GAA gene mutation analysis
  • ✓Form specification: infantile-onset, late-onset, or childhood-onset
  • ✓Cardiac evaluation including echocardiogram findings if infantile form
  • ✓Pulmonary function testing documenting respiratory muscle involvement
  • ✓Enzyme replacement therapy status and response to treatment

Commonly Confused Codes

  • •E74.00 — Glycogen storage disease, unspecified: do not use when Pompe disease is documented
  • •E74.05 — LAMP2 deficiency (Danon disease): also causes cardiomyopathy with glycogen accumulation but is a different enzyme
  • •E74.04 — McArdle disease: muscle-only GSD but involves myophosphorylase, not acid maltase
  • •I42.8 — Other cardiomyopathies: cardiomyopathy is a manifestation of Pompe, code the underlying cause
  • •G72.9 — Myopathy, unspecified: muscle weakness is a symptom, not the primary diagnosis

Child Codes

E74.00Glycogen storage disease, unspecified
HCC 50
E74.01von Gierke disease
HCC 50
E74.02Pompe disease
HCC 49
E74.03Cori disease
HCC 50
E74.04McArdle disease
HCC 50
E74.05Lysosome-associated membrane protein 2 [LAMP2] deficiency
HCC 50
E74.09Other glycogen storage disease
HCC 50

Code Hierarchy

└E74Other disorders of carbohydrate metabolism└E74.0Glycogen storage disease└E74.02Pompe disease
└E74.02Pompe disease

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