2027 ICD-10-CM Diagnosis Code E75.21Fabry (-Anderson) disease
ICD-10-CM Codes›E00–E89›E70-E88›E75
- Billable — Valid for Submission
- Risk Adjusts — HCC 49
- Chronic Condition
E75.21 is a billable ICD-10-CM diagnosis code for fabry (-Anderson) disease. It is valid on HIPAA claims for fiscal year 2027 (October 1, 2026 through September 30, 2027) and groups to MS-DRG 642. Coders also document this condition as autonomic neuropathy due to Fabry disease. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Other specified and unspecified nutritional and metabolic disorders.
For Medicare Advantage risk adjustment, E75.21 maps to CMS-HCC Category 49 (Specified Lysosomal Storage Disorders) under the V28 model, adding a risk factor of about 9.256 for a community, non-dual, aged beneficiary in payment year 2027.
Code Identity
Code Classification
Medicare Risk Adjustment (HCC)Billing
E75.21 maps to a payment category in the CMS-HCC model used to risk-adjust Medicare Advantage payments. Weights are the published community factors for payment year 2027.
Source: CMS Payment Year 2027 risk adjustment mappings and model software. Weights are relative factors, not dollar amounts; a beneficiary's total RAF also includes demographics and interactions. Browse all CMS-HCC categories.
Approximate SynonymsGuidance
Alternate terms and clinical phrases that map to this code.
- Autonomic neuropathy due to Fabry disease
- Deficiency of alpha-galactosidase
- Fabry's disease
- Glomerular disease due to Fabry disease
- Kidney disease due to genetic disorder of lipid storage
Instructional NotesGuidance
Instructions from the official ICD-10-CM Tabular List that apply to E75.21: its own notes plus those printed at E75.2, E75, block E70-E88, and Chapter 4. A note printed at a category, block or chapter applies to every code under it.
Excludes1
Not coded here: the excluded code is never reported together with this one, unless the two conditions are unrelated.
- adrenoleukodystrophy [Addison-Schilder] (E71.528)
Excludes2
Not included here: the excluded condition is not part of this code, but a patient may have both, so both codes may be reported.
- Ehlers-Danlos syndromes (Q79.6-)
Notes
General instructions on how these codes are used.
- All neoplasms, whether functionally active or not, are classified in Chapter 2. Appropriate codes in this chapter (i.e. E05.8, E07.0, E16-E31, E34.-) may be used as additional codes to indicate either functional activity by neoplasms and ectopic endocrine tissue or hyperfunction and hypofunction of endocrine glands associated with neoplasms and other conditions classified elsewhere.
Source: CMS ICD-10-CM Tabular List. How to read instructional notes.
Referenced in Other NotesGuidance
Instructional notes printed at other codes that name E75.21, its category, or a range that includes it.
Excludes1 2
These codes carry an Excludes1 note naming E75.21: they are not reported together with it, unless the two conditions are unrelated.
Code First 2
These codes say to code first a condition named in the note, and E75.21 is one of the conditions named.
Index to Diseases and InjuriesGuidance
Alphabetical index entries that point to this code.
Anderson-Fabry disease E75.21
Angiokeratoma See: Neoplasm, skin, benign;
corporis diffusum E75.21
Disease, diseased See Also: Syndrome;
Sweeley-Klionsky E75.21
Disorder (of) See Also: Disease;
Fabry's disease E75.21
Fabry (-Anderson) disease E75.21
Glomerulonephritis See Also: Nephritis; N05.9
glycolipid E75.21
Sweeley-Klionsky disease E75.21
Clinical ClassificationClinical
AHRQ’s CCSR groups this code into broader clinical categories.
Clinical InformationClinical
Fabry Disease
an x-linked inherited metabolic disease caused by a deficiency of lysosomal alpha-galactosidase a. it is characterized by intralysosomal accumulation of globotriaosylceramide and other glycosphingolipids in blood vessels throughout the body leading to multi-system complications including renal, cardiac, cerebrovascular, and skin disorders.
Patient EducationClinical
Genetic Brain Disorders
A genetic brain disorder is caused by a variation or a mutation in a gene. A variation is a different form of a gene. A mutation is a change in a gene. Genetic brain disorders affect the development and function of the brain.
Read the full article at MedlinePlus
Courtesy of MedlinePlus, a service of the U.S. National Library of Medicine.
Convert E75.21 to ICD-9-CMHistory
The closest ICD-9-CM equivalents under the General Equivalence Mappings.
Code HistoryHistory
Questions About E75.21Overview
What is the ICD-10 code for fabry (-Anderson) disease?
The ICD-10-CM code for fabry (-Anderson) disease is E75.21 (sometimes written as E7521). It is billable on HIPAA-covered claims from October 1, 2026 through September 30, 2027.
Is E75.21 (Other sphingolipidosis) a billable code?
Yes. This is a billable ICD-10-CM code, specific enough to report fabry (-Anderson) disease on HIPAA-covered claims from October 1, 2026 through September 30, 2027.
What MS-DRG does E75.21 group to?
When fabry (-Anderson) disease is the principal diagnosis on an inpatient stay, it groups to MS-DRG 642 (inborn and Other Disorders of Metabolism), which carries a relative weight of 1.3226. Higher weights mean higher Medicare reimbursement.
Can E75.21 be reported with E71.528?
Not as a rule. The Excludes1 note at E75.2, which applies to E75.21, lists "adrenoleukodystrophy [Addison-Schilder] (E71.528)". An Excludes1 note means the excluded code is never reported together with this one; the only exception is when the two conditions are unrelated to each other (Official Guidelines, Section I.A.12.a).
What is the ICD-9 equivalent of E75.21?
Under the General Equivalence Mappings, fabry (-Anderson) disease converts to ICD-9-CM 272.7 (lipidoses). The mapping is approximate, so confirm the match fits the documentation.
What HCC is E75.21?
E75.21 (fabry (-Anderson) disease) maps to CMS-HCC Category 49 (Specified Lysosomal Storage Disorders), commonly written as HCC 49, in the CMS-HCC V28 model used for Medicare Advantage risk adjustment in payment year 2027. It mapped to HCC 23 under the retired V24 model. It also maps in the PACE (CMS-HCC V22), ESRD (V21), and ESRD (V24) models. In the Part D prescription drug model it maps to RxHCC 41.
Does E75.21 risk-adjust for Medicare Advantage payment?
Yes. When documented and reported on a Medicare Advantage encounter, E75.21 adds a risk adjustment factor of about 9.256 to the beneficiary's RAF score for a community, non-dual, aged enrollee (published V28 weights range from 1.528 to 13.778 depending on the payment segment). HCC 49 sits at the top of its hierarchy, so no other condition category supersedes it. See the full factor table on the HCC 49 category page.
