The U.S. Center for Medicare and Medicaid (CMS) has updated their list of diagnostic codes, known as the International Classification of Diseases Version 10 (ICD-10), with two notable additions:
- N07.B Hereditary nephropathy, not elsewhere classified with APOL1-mediated kidney disease (AMKD)
- Z84.11 Family history of APOL1-mediated kidney disease (AMKD)
The availability of these codes is a significant advance for a disease that was only identified in 2010. The codes will make people living with AMKD or with a family history visible within the healthcare system. This has the potential to transform patient care and expand research capabilities for potential treatments.
Understanding AMKD
AMKD is a rapidly progressive, proteinuric form of chronic kidney disease caused by two variants in the APOL1 gene. It occurs in people of African ancestry, which can include people who identify as Black, African, African American, Latino/a and/or Afro-Caribbean.
All humans carry the APOL1 gene, which is part of the immune defense against parasitic infections. The G1 and G2 APOL1 variants evolved in central and western Africa to defend against resistant trypanosomes in that region. Carrying an APOL1 variant presented a survival advantage; however, carrying two copies brought about increased risk of kidney disease. APOL1 genetic variants are toxic to several cell lines in the kidney, leading to glomerular injury that presents as proteinuria and loss of kidney function.
AMKD affects an estimated patient population of approximately 250,000 in the U.S. and Europe. There are no therapies currently approved for AMKD.
Learn more about our work in AMKD.
Why this matters for AMKD patients and their healthcare team
Properly recording AMKD diagnoses and family history allows for broader recognition of the condition, supports accurate billing practices and helps to raise awareness of kidney health among those with a family history of the disease.
Previously, patients with AMKD were diagnosed and documented using the code for chronic kidney disease, which does not identify cause. A precise diagnosis of this genetic kidney disease may improve care of both the patient and family members at risk, facilitating genetic testing, counseling and early intervention.
The option to code for family history of AMKD can improve health monitoring and management in affected families. Awareness of heightened AMKD risk may enhance screening and preventative care, which may improve outcomes. In addition, using the code for family history may provide rationale for ordering genetic testing.
What are ICD-10 codes?
The ICD-10, managed and published by the World Health Organization (WHO), is a set of codes used to classify and code diseases, symptoms, and procedures. It creates a common language that is used in insurance billing, research, and patient care. In the U.S., we use the ICD-10 Clinical Modification (ICD-10-CM), which is revised every fiscal year to provide more specificity for disease classification and reporting in U.S. healthcare settings. The addition of a condition to the ICD-10-CM facilitates specificity and improved accuracy in patient diagnoses, treatment options, and healthcare coverage.
Starting October 1, 2025, AMKD may be documented precisely, communicating presence of this rapidly progressive, proteinuric genetic kidney disease to all care providers.
The creation of these codes is the result of a collaborative effort by the kidney community, led by the Renal Physicians Association.
Learn more about AMKD:
· Educated and empowered: Emani’s story
· Disease awareness for HCPs: What is AMKD?
· Genetic testing as a tool for kidney health
· A nephrologist’s perspective on APOL1-mediated kidney disease