Nephrotic syndrome (N04) ICD-10-CM
The N04 code range covers nephrotic syndrome with 19 ICD-10-CM diagnosis codes. 16 of them are billable and valid for claim submission in fiscal year 2026, and the category headers group them but cannot themselves be billed.
Includes
This note appears immediately under a three character code title to further define, or give examples of, the content of the category.
- congenital nephrotic syndrome
- lipoid nephrosis
Codes in the N04 Range 19 codes · 16 billable
- N04 Nephrotic syndromeNon-billable
- N04.0 Nephrotic syndrome with minor glomerular abnormality
- N04.1 Nephrotic syndrome with focal and segmental glomerular lesions
- N04.2 Nephrotic syndrome with diffuse membranous glomerulonephritisNon-billable
- N04.20 Nephrotic syndrome with diffuse membranous glomerulonephritis, unspecified
- N04.21 Primary membranous nephropathy with nephrotic syndrome
- N04.22 Secondary membranous nephropathy with nephrotic syndrome
- N04.29 Other nephrotic syndrome with diffuse membranous glomerulonephritis
- N04.3 Nephrotic syndrome with diffuse mesangial proliferative glomerulonephritis
- N04.4 Nephrotic syndrome with diffuse endocapillary proliferative glomerulonephritis
- N04.5 Nephrotic syndrome with diffuse mesangiocapillary glomerulonephritis
- N04.6 Nephrotic syndrome with dense deposit disease
- N04.7 Nephrotic syndrome with diffuse crescentic glomerulonephritis
- N04.8 Nephrotic syndrome with other morphologic changes
- N04.9 Nephrotic syndrome with unspecified morphologic changes
- N04.A Nephrotic syndrome with C3 glomerulonephritis
- N04.B Nephrotic syndrome with immune complex membranoproliferative glomerulonephritis (IC-MPGN)Non-billable
- N04.B1 Nephrotic syndrome with idiopathic immune complex membranoproliferative glomerulonephritis (IC-MPGN) New
- N04.B2 Nephrotic syndrome with secondary immune complex membranoproliferative glomerulonephritis (IC-MPGN) New
Clinical Terms in This Code Range
Definitions from the National Library of Medicine for conditions coded in the N04 range.
Nephrosis, Lipoid
A kidney disease with no or minimal histological glomerular changes on light microscopy and with no immune deposits. It is characterized by lipid accumulation in the epithelial cells of KIDNEY TUBULES and in the URINE. Patients usually show NEPHROTIC SYNDROME indicating the presence of PROTEINURIA with accompanying EDEMA.
Nephrotic Syndrome
A condition characterized by severe PROTEINURIA, greater than 3.5 g/day in an average adult. The substantial loss of protein in the urine results in complications such as HYPOPROTEINEMIA; generalized EDEMA; HYPERTENSION; and HYPERLIPIDEMIAS. Diseases associated with nephrotic syndrome generally cause chronic kidney dysfunction.
About the N04 Code Range
The ICD-10 code section N04 covers nephrotic syndrome, a kidney disorder causing excess protein loss in urine. These codes specify different types and underlying causes of nephrotic syndrome to help accurately capture patient diagnoses.
This code range includes various specific forms such as N04.0 for nephrotic syndrome with minor glomerular abnormalities, also known as minimal change glomerulonephritis, and N04.1 for cases involving focal and segmental glomerulosclerosis (FSGS). The codes N04.2 through N04.29 refer to nephrotic syndrome with membranous glomerulonephritis, with subcategories for primary and secondary membranous nephropathy. Other notable codes include N04.3 for diffuse mesangial proliferative glomerulonephritis and N04.5 for diffuse mesangiocapillary (membranoproliferative) glomerulonephritis. The N04.9 code covers nephrotic syndrome cases with unspecified or varied morphologic changes, including steroid-sensitive and steroid-resistant forms, as well as syndromes associated with congenital or systemic diseases. Knowing the ICD-10 code for nephrotic syndrome is essential for coding accurate clinical details for diagnosis and treatment planning.
Questions About This Page
How many billable codes are in the N04 range?
Of the 19 codes in this range, 16 are billable and valid for claim submission from October 1, 2025 through September 30, 2026. Category header codes group them but cannot be reported on claims.
What does the N04 range classify?
The range classifies nephrotic syndrome. Each code links to its own reference page with billing status, MS-DRG grouping, coding notes, and clinical information.
Related References
Source: CMS FY 2026 ICD-10-CM Tabular List and order file, effective October 1, 2025 through September 30, 2026.
