2026 ICD-10-CM Diagnosis Code N04.8Nephrotic syndrome with other morphologic changes

ICD-10-CM Codes›N00–N99›N00-N08›N04

ICD-10-CM N04.8
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

N04.8 is a billable ICD-10-CM diagnosis code for nephrotic syndrome with other morphologic changes. It is valid on HIPAA claims for fiscal year 2026 (October 1, 2025 through September 30, 2026) and groups to MS-DRG 698 through 700. Coders also document this condition as congenital nephrotic syndrome. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Nephritis; nephrosis; renal sclerosis.

Code Identity

ICD-10-CM Code
N04.8
Billable Status
Yes — Valid for Submission
Code Describes
Nephrotic syndrome with other morphologic changes
Short Description
Nephrotic syndrome with other morphologic changes
Same as the full description in the CMS dataset.
Parent Code
N04
Nephrotic syndrome

Code Classification

ChapterN00–N99Diseases of the genitourinary system
SectionN00-N08Glomerular diseases
CategoryN04Nephrotic syndrome
This CodeN04.8Nephrotic syndrome with other morphologic changes

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Congenital nephrotic syndrome
  • Drash syndrome
  • Finnish congenital nephrotic syndrome
  • Glomerular disease due to Finnish type congenital nephrotic syndrome
  • Nephrotic syndrome with proliferative glomerulonephritis

Tabular List NotesGuidance

Coding notes and annotation back-references applicable to this code.

Inclusion Terms

  • Nephrotic syndrome with proliferative glomerulonephritis NOS

Index to Diseases and InjuriesGuidance

Alphabetical index entries that point to this code.

External Cause of Injuries IndexGuidance

References for this code in the External Cause of Injuries Index.

    • Syndrome
      • nephrotic (congenital)
        • with
          • specified morphological changes NEC

Clinical ClassificationClinical

AHRQ’s CCSR groups this code into broader clinical categories.

CCSR GEN001
Nephritis; nephrosis; renal sclerosis
Default principal diagnosis: inpatient Yes · outpatient Yes

Clinical InformationClinical

  • Congenital Nephrotic Syndrome

    a rare autosomal recessive inherited nephrotic syndrome that is present in the first week of life. it manifests with edema and proteinuria and usually has a poor prognosis.

  • Congenital Nephrotic Syndrome - Cytomegalovirus Associated|Cytomegalovirus Associated Congenital Nephrotic Syndrome

    nephrotic syndrome associated with a cytomegalovirus infection, most commonly presenting in the first three months of life.

  • Congenital Nephrotic Syndrome - Diffuse Mesangial Sclerosis|Diffuse Mesangial Sclerosis|Diffuse Mesangial Sclerosis

    nephrotic syndrome within the first three motnhs of life, characterized initially by increased mesangial matrix, with or without hypertrophy and hyperplasia of podocytes, and eventual glomerular sclerosis.

  • Congenital Nephrotic Syndrome - Focal Segmental Glomerulosclerosis|Congenital FSGS|Congenital FSGS

    nephrotic syndrome within the first three months of life, characterized by scarring of the glomerulus, in which only part of the glomerulus is involved, and less than 50% of the glomeruli are affected.

  • Congenital Nephrotic Syndrome - Infection Associated|Infection Associated Congenital Nephrotic Syndrome

    nephrotic syndrome presenting within the first three months of life, and which is associated with an infectious process.

  • Congenital Nephrotic Syndrome - Rubivirus Associated|Rubivirus Associated Congenital Nephrotic Syndrome

    nephrotic syndrome associated with rubella, most commonly presenting in the first three months of life.

  • Congenital Nephrotic Syndrome - Toxoplasma Associated|Toxoplasma Associated Congenital Nephrotic Syndrome

    nephrotic syndrome associated with toxoplasmosis, most commonly presenting in the first three months of life.

  • Congenital Nephrotic Syndrome - Treponema Pallidum Associated|Treponema Pallidum Associated Congenital Nephrotic Syndrome

    nephrotic syndrome associated with syphilis, most commonly presenting in the first three months of life.

  • Nephrotic Syndrome - NPHS1 Associated|Congenital Nephrotic Syndrome - Finnish Type

    nephrotic syndrome attributed to mutation(s) in the nphs1 gene, which encodes the protein nephrin, and most commonly presents during the first three months of life.

Patient EducationClinical

Kidney Diseases

You have two kidneys, each about the size of your fist. They are near the middle of your back, just below the rib cage. Inside each kidney there are about a million tiny structures called nephrons. They filter your blood. They remove wastes and extra water, which become urine. The urine flows through tubes called ureters.

Read the full article at MedlinePlus

Courtesy of MedlinePlus, a service of the U.S. National Library of Medicine.

Convert N04.8 to ICD-9-CMHistory

The closest ICD-9-CM equivalents under the General Equivalence Mappings.

ICD-9-CM
581.89 Nephrotic syndrome NEC
Approximate The match is approximate rather than exact.

Code History & ChangesHistory

Replaced This code was replaced in the FY 2026 code set by:

  • N04.B1 - Nep syn w idio imm cmplx membr prolif glomrlneph (IC-MPGN)
  • N04.B1 - Nep syn w idio imm cmplx membr prolif glomrlneph (IC-MPGN)
  • N04.B2 - Nep syn with sec imm cmplx membr prolif glomrlneph (IC-MPGN)
  • N04.B2 - Nep syn with sec imm cmplx membr prolif glomrlneph (IC-MPGN)
FY 2016AddedAdded to the ICD-10-CM code setEffective October 1, 2015, the first year of ICD-10-CM.
FY 2017–2025No changes
FY 2026CurrentCurrent code set, no changesEffective October 1, 2025 through September 30, 2026.

Questions About N04.8Overview

Is N04.8 (Nephrotic syndrome) a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report nephrotic syndrome with other morphologic changes on HIPAA-covered claims from October 1, 2025 through September 30, 2026.

What MS-DRG does N04.8 group to?

When nephrotic syndrome with other morphologic changes is the principal diagnosis on an inpatient stay, it groups to MS-DRG 698, 699, 700, with relative weights from 0.6899 to 1.6544 depending on complications. Higher weights mean higher Medicare reimbursement.

What is the ICD-9 equivalent of N04.8?

Under the General Equivalence Mappings, nephrotic syndrome with other morphologic changes converts to ICD-9-CM 581.89 (nephrotic syndrome NEC). The mapping is approximate, so confirm the match fits the documentation.

Footnotes

[1] Chronic - a chronic condition code indicates a condition lasting 12 months or longer and its effect on the patient based on one or both of the following criteria:

  • The condition results in the need for ongoing intervention with medical products,treatment, services, and special equipment
  • The condition places limitations on self-care, independent living, and social interactions.