ICD-10-CM Code N04

Nephrotic syndrome

Version 2020 Non-Billable Code

Not Valid for Submission

N04 is a "header" nonspecific and non-billable code code, consider using a code with a higher level of specificity for a diagnosis of nephrotic syndrome. The code is NOT valid for the year 2020 for the submission of HIPAA-covered transactions.

Short Description:Nephrotic syndrome
Long Description:Nephrotic syndrome

Consider the following ICD-10 codes with a higher level of specificity:

  • 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 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

Tabular List of Diseases and Injuries

The Tabular List of Diseases and Injuries is a list of ICD-10 codes, organized "head to toe" into chapters and sections with guidance for inclusions, exclusions, descriptions and more. The following references are applicable to the code N04:


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

Clinical Information

  • 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.

Code Classification

  • Diseases of the genitourinary system (N00–N99)
    • Glomerular diseases (N00-N08)
      • Nephrotic syndrome (N04)

Code History

  • FY 2016 - New Code, effective from 10/1/2015 through 9/30/2016
    (First year ICD-10-CM implemented into the HIPAA code set)
  • FY 2017 - No Change, effective from 10/1/2016 through 9/30/2017
  • FY 2018 - No Change, effective from 10/1/2017 through 9/30/2018
  • FY 2019 - No Change, effective from 10/1/2018 through 9/30/2019
  • FY 2020 - No Change, effective from 10/1/2019 through 9/30/2020

Information for Patients

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. It goes to your bladder, which stores the urine until you go to the bathroom.

Most kidney diseases attack the nephrons. This damage may leave kidneys unable to remove wastes. Causes can include genetic problems, injuries, or medicines. You have a higher risk of kidney disease if you have diabetes, high blood pressure, or a close family member with kidney disease. Chronic kidney disease damages the nephrons slowly over several years. Other kidney problems include

  • Cancer
  • Cysts
  • Stones
  • Infections

Your doctor can do blood and urine tests to check if you have kidney disease. If your kidneys fail, you will need dialysis or a kidney transplant.

NIH: National Institute of Diabetes and Digestive and Kidney Diseases

[Learn More]

Congenital nephrotic syndrome Congenital nephrotic syndrome is a kidney condition that begins in infancy and typically leads to irreversible kidney failure (end-stage renal disease) by early childhood. Children with congenital nephrotic syndrome begin to have symptoms of the condition between birth and 3 months.The features of congenital nephrotic syndrome are caused by failure of the kidneys to filter waste products from the blood and remove them in urine. Signs and symptoms of this condition are excessive protein in the urine (proteinuria), increased cholesterol in the blood (hypercholesterolemia), an abnormal buildup of fluid in the abdominal cavity (ascites), and swelling (edema). Affected individuals may also have blood in the urine (hematuria), which can lead to a reduced number of red blood cells (anemia) in the body, abnormal blood clotting, or reduced amounts of certain white blood cells. Low white blood cell counts can lead to a weakened immune system and frequent infections in people with congenital nephrotic syndrome.Children with congenital nephrotic syndrome typically develop end-stage renal disease between ages 2 and 8, although with treatment, some may not have kidney failure until adolescence or early adulthood.
[Learn More]