ICD-10-CM 2027
ICD-10 code for Sweeley-Klionsky disease
From the official ICD-10-CM alphabetic index entry “Sweeley-Klionsky disease”. Page updated September 29, 2026.
About coding Sweeley-Klionsky disease
The ICD-10-CM code for Sweeley-Klionsky disease is E75.21 (Fabry (-Anderson) disease).
Within E75.2 (other sphingolipidosis), E75.21 is specifically for Fabry (-Anderson) disease. Related codes cover Gaucher disease (E75.22), Krabbe disease (E75.23), Niemann-Pick disease (E75.24), Metachromatic leukodystrophy (E75.25), Sulfatase deficiency (E75.26), Pelizaeus-Merzbacher disease (E75.27), Canavan disease (E75.28) and other sphingolipidosis (E75.29).
It is not a CC or MCC, so as a secondary diagnosis it doesn't change the MS-DRG severity level.
In MS-DRG v44.0, it is part of the grouping logic for DRG 642 (Inborn and Other Disorders of Metabolism, relative weight 1.3219), in MDC 10 (Endocrine, Nutritional and Metabolic Diseases and Disorders), as a principal or secondary diagnosis. The DRG a claim lands in depends on the principal diagnosis, procedures and any CC/MCC secondary diagnoses.
Common questions about Sweeley-Klionsky disease ICD-10 codes
What is the ICD-10 code for Sweeley-Klionsky disease?
- E75.21 — Fabry (-Anderson) disease.
Is E75.21 billable?
- Yes. E75.21 is a billable/specific ICD-10-CM code valid for FY2027 (October 1, 2026 – September 30, 2027).
Can E75.21 be used as a principal diagnosis?
- Yes. The Medicare Code Editor doesn't restrict E75.21 as a principal diagnosis.
Is E75.21 a CC or MCC?
- No. E75.21 is neither a CC nor an MCC under MS-DRG v44.0.