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ICD-10 Guide
DiagnosesAlport Syndrome

Alport Syndrome

ICD-10 Coding for Alport Syndrome(Q87.81)

PRIMARY SPECIALTYNephrology
COMPLEXITYHigh
LAST UPDATED09/15/2025
Sam Tuffun, PT, DPT
Physical Therapist | Medical Coding & Billing Contributor

Diagnosis Overview

What is Alport Syndrome?
Essential facts and insights about Alport Syndrome

Key Clinical Considerations:

  • Hematuria (blood in urine), proteinuria (excess protein in urine), and progressive renal insufficiency.
  • Genetic testing revealing mutations in the COL4A3, COL4A4, or COL4A5 genes.
  • Physical examination may show signs of renal failure, such as hypertension or edema.
  • Imaging studies may reveal renal abnormalities, but are not definitive for diagnosis.
  • Severity is often assessed by the degree of renal impairment and the presence of extrarenal manifestations such as hearing loss or ocular abnormalities.

Clinical Information

Clinical Criteria & Documentation Requirements

  • Document the presence of hematuria and proteinuria in the medical record.
  • Use specific terminology such as 'Alport Syndrome' and 'glomerular basement membrane disorder'.
  • Examples include: 'Patient presents with hematuria and proteinuria consistent with Alport Syndrome.'
  • Document medical necessity for genetic testing and any referrals to specialists.
  • Quality measures may include tracking renal function and monitoring for complications.

Coding Guidelines

Usage Guidelines & Examples

  • Use this diagnosis code when a patient presents with classic symptoms of Alport Syndrome and confirmed genetic mutations.
  • Do not use this code for patients with isolated hematuria without other symptoms or genetic confirmation.
  • Compare with codes for other hereditary nephropathies, such as Focal Segmental Glomerulosclerosis (N18.30).
  • Common errors include misclassifying Alport Syndrome as a primary glomerulonephritis without genetic confirmation.
  • In complex cases, ensure all clinical findings and genetic testing results are documented to support code selection.

Code Exclusions

Important Exclusions

  • Exclude codes for other types of nephritis that do not involve genetic mutations.
  • Alternative codes for excluded conditions may include N17 (Acute kidney failure) or N18 (Chronic kidney disease).
  • Conditions are excluded due to differing pathophysiology and treatment approaches.
  • Common mistakes include misdiagnosing Alport Syndrome in patients with unrelated renal conditions.
  • Related but distinct conditions include IgA nephropathy and thin basement membrane nephropathy.

Related ICD-10 Codes

Primary Codes
Q87.81
Alport syndrome
N18.9
Chronic kidney disease, unspecified
Ancillary Codes
N04.9
H91.3
H27.1
Differential Codes
N04.9
H91.3

Related CPT Codes

CPT codes will be available in a future update.

Specialty Focus

Primary Specialty

Nephrology

Specialty Applications

  • Applies to patients with hereditary kidney disorders, particularly those with a family history of renal disease.
  • Patient populations include children and young adults, with a higher prevalence in males.
  • Clinical settings include nephrology clinics, genetic counseling, and pediatric care.
  • Specialty-specific applications are relevant in nephrology and genetics.
  • Treatment contexts include management of renal function and monitoring for complications.

Coding Complexity

High Complexity

This diagnosis requires careful attention to:

  • Comprehensive clinical documentation
  • Accurate code selection based on clinical criteria
  • Proper exclusion considerations
  • Specialty-specific coding guidelines

Documentation

Documentation Templates

Template 1

Template: 'Patient diagnosed with Alport Syndrome based on clinical findings of hematuria and proteinuria.'

Template 2

Template: 'Clinical presentation consistent with Alport Syndrome including renal failure and hearing loss.'

Template 3

Template: 'Diagnostic criteria for Alport Syndrome met as evidenced by genetic testing results.'

Template 4

Template: 'Treatment plan initiated for Alport Syndrome with nephrology referral and monitoring.'

Template 5

Template: 'Follow-up care for Alport Syndrome including regular renal function tests and audiology assessments.'

Billing Information

Billing Considerations

  • Ensure proper documentation for billing
  • Verify code specificity requirements
  • Check for any additional codes needed
  • Review payer-specific guidelines

Common Issues

  • Insufficient clinical documentation
  • Incorrect code selection
  • Missing supporting diagnoses
  • Timing and frequency documentation

Frequently Asked Questions

What documentation is required for this diagnosis?

Document clinical findings such as hematuria, proteinuria, and genetic test results.

How does this differ from similar diagnoses?

Alport Syndrome is characterized by specific genetic mutations and associated extrarenal symptoms.

What are common billing considerations?

Ensure that all services related to genetic testing and nephrology consultations are documented for reimbursement.

What procedures are typically associated?

Related CPT codes may include renal biopsy and genetic testing procedures.

Are there any quality reporting implications?

Quality measures may include tracking renal function and patient outcomes related to Alport Syndrome.