E74.02 Pompe disease

The ICD-10-CM code for Pompe disease is E74.02 (FY2026). It is a billable, claim-ready diagnosis code.

Classification

Chapter

4: Endocrine, nutritional and metabolic diseases (E00-E89)

Section

E70-E88: Metabolic disorders (E70-E88)

Category E74

32 codes (24 billable)

FY2026 Status

Stable since FY2024

Also Known As

ICD-10-CM Alphabetic Index entries that lead to E74.02:

• Pompe's disease (glycogen storage)
• Cardiomegalia glycogenica diffusa
• Glycogenosis (diffuse) (generalized) › cardiac
• Cardiomegaly › glycogen
• Cardiomyopathy (familial) (idiopathic) › glycogen storage
• Deficiency, deficient › lysosomal alpha-1, 4 glucosidase
• Disease, diseased › Pompe's (glycogenosis II)
• Myocardiopathy (congestive) (constrictive) (familial) (hypertrophic nonobstructive) (idiopathic) (infiltrative) (obstructive) (primary) (restrictive) (sporadic) › glycogen storage

Inclusion Terms

• Cardiac glycogenosis
• Type II glycogen storage disease

U.S. Hospital Utilization

• An estimated 465 U.S. inpatient stays in 2023 included E74.02 among the documented diagnoses.
• 85 stays listed it as the principal diagnosis.

Source: National Inpatient Sample (NIS), Healthcare Cost and Utilization Project (HCUP), Agency for Healthcare Research and Quality, 2016–2023. National survey-weighted estimates.

Official Coding Guidelines

Diabetes mellitus (TOC entry)

a. Diabetes mellitus ................................................................................................................... 39

Obesity (TOC entry)

b. Obesity .................................................................................................................................. 42

Source: CMS — ICD-10-CM Official Guidelines for Coding and Reporting, FY2026

References

• CMS — ICD-10-CM Official Code Set and Guidelines, FY2026
• CDC/NCHS — ICD-10-CM Files and Browser Tool
• AHRQ — HCUP National Inpatient Sample, Frequencies by Diagnosis Code (2016–2023)