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ICD-10 Guide
ICD-10 CodesN01.4

N01.4

Billable

Rapidly progressive nephritic syndrome with diffuse endocapillary proliferative glomerulonephritis

BILLABLE STATUSYes
IMPLEMENTATION DATEOctober 1, 2015
LAST UPDATED09/18/2025

Code Description

ICD-10 N01.4 is a billable code used to indicate a diagnosis of rapidly progressive nephritic syndrome with diffuse endocapillary proliferative glomerulonephritis.

Key Diagnostic Point:

Rapidly progressive nephritic syndrome (RPNS) is characterized by a swift decline in renal function, often leading to end-stage renal disease if not promptly treated. This syndrome is associated with diffuse endocapillary proliferative glomerulonephritis, which is marked by the proliferation of glomerular endothelial cells and inflammatory cells within the capillary loops. Clinically, patients present with symptoms of nephritis, including hematuria, proteinuria, and hypertension. Laboratory findings typically reveal significant proteinuria, often exceeding 3.5 grams per day, and the presence of red blood cell casts in the urine. Renal biopsy is crucial for diagnosis, revealing glomerular hypercellularity and often immune complex deposition. Management of RPNS involves addressing the underlying cause, which may include immunosuppressive therapy, corticosteroids, and supportive care for renal function. Early intervention is critical to prevent irreversible kidney damage.

Code Complexity Analysis

Complexity Rating: High

High Complexity

Complexity Factors

  • Requires understanding of nephrology terminology and conditions
  • Differentiation from other types of glomerulonephritis
  • Need for precise documentation of clinical findings
  • Potential for multiple underlying causes

Audit Risk Factors

  • Inadequate documentation of clinical findings
  • Failure to specify the type of glomerulonephritis
  • Misclassification of nephritic vs. nephrotic syndrome
  • Lack of supporting lab results

Specialty Focus

Medical Specialties

Nephrology

Documentation Requirements

Detailed clinical notes on symptoms, lab results, and treatment plans.

Common Clinical Scenarios

Patients presenting with acute kidney injury, hematuria, and proteinuria.

Billing Considerations

Ensure all relevant lab results and imaging studies are documented to support the diagnosis.

Pathology

Documentation Requirements

Comprehensive reports on renal biopsy findings, including histological details.

Common Clinical Scenarios

Biopsy evaluations for suspected glomerulonephritis.

Billing Considerations

Pathology reports should clearly indicate the type of glomerular disease to support nephrology coding.

Coding Guidelines

Inclusion Criteria

Use N01.4 When
  • Follow the official ICD
  • CM coding guidelines, ensuring accurate documentation of the clinical picture
  • Include specific details about the type of nephritis and any associated conditions

Exclusion Criteria

Do NOT use N01.4 When
  • Exclude codes that do not reflect the rapidly progressive nature of the syndrome

Related ICD-10 Codes

Related CPT Codes

88172CPT Code

Renal biopsy, percutaneous

Clinical Scenario

Used when a renal biopsy is performed to confirm diagnosis.

Documentation Requirements

Document indication for biopsy, findings, and any complications.

Specialty Considerations

Nephrology specialists should ensure biopsy results are linked to the diagnosis.

ICD-10 Impact

Diagnostic & Documentation Impact

Enhanced Specificity

ICD-10 Improvements

The transition to ICD-10 has allowed for more specific coding of nephritic syndromes, improving the accuracy of data collection and reimbursement processes. N01.4 provides a clear distinction for rapidly progressive cases, which is critical for appropriate management and resource allocation.

ICD-9 vs ICD-10

The transition to ICD-10 has allowed for more specific coding of nephritic syndromes, improving the accuracy of data collection and reimbursement processes. N01.4 provides a clear distinction for rapidly progressive cases, which is critical for appropriate management and resource allocation.

Reimbursement & Billing Impact

reimbursement processes. N01.4 provides a clear distinction for rapidly progressive cases, which is critical for appropriate management and resource allocation.

Resources

Clinical References

  • •
    American Academy of Nephrology

Coding & Billing References

  • •
    American Academy of Nephrology

Frequently Asked Questions

What are the key symptoms of rapidly progressive nephritic syndrome?

Key symptoms include hematuria, proteinuria, hypertension, and rapid decline in renal function.

How is rapidly progressive nephritic syndrome diagnosed?

Diagnosis is typically made through clinical evaluation, laboratory tests showing proteinuria and hematuria, and renal biopsy confirming glomerular pathology.