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Nephrotic Syndrome

Nephrotic Syndrome
Author: Eric P Cohen, MD; Chief Editor: Vecihi Batuman, MD, FACP , FASN more...
Updated: Dec 9, 2014

Background
Nephrotic syndrome is kidney disease with proteinuria, hypoalbuminemia, and edema. Nephrotic-range proteinuria
is 3 grams per day or more. On a single spot urine collection, it is 2 g of protein per gram of urine creatinine.
There are many specific causes of nephrotic syndrome . These include kidney diseases such as minimal-change
nephropathy, focal glomerulosclerosis, and membranous nephropathy. Nephrotic syndrome can also result from
systemic diseases that affect other organs in addition to the kidneys, such as diabetes, amyloidosis, and lupus
erythematosus.
Nephrotic syndrome may affect adults and children, of both sexes and of any race. It may occur in typical form, or in
association with nephritic syndrome. The latter connotes glomerular inflammation, with hematuria and impaired
kidney function.

Classification
Nephrotic syndrome can be primary, being a disease specific to the kidneys, or it can be secondary, being a renal
manifestation of a systemic general illness. In all cases, injury to glomeruli is an essential feature.
Primary causes of nephrotic syndrome include the following, in approximate order of frequency:
Minimal-change nephropathy
Focal glomerulosclerosis
Membranous nephropathy
Hereditary nephropathies
Secondary causes include the following, again in order of approximate frequency:
Diabetes mellitus
Lupus erythematosus
Amyloidosis and paraproteinemias
Viral infections (eg, hepatitis B, hepatitis C, human immunodeficiency virus [HIV] )
Preeclampsia
Nephrotic-range proteinuria may occur in other kidney diseases, such as IgA nephropathy. In that common
glomerular disease , one third of patients may have nephrotic-range proteinuria . [1]
Nephrotic syndrome may occur in persons with sickle cell disease and evolve to renal failure. Membranous
nephropathy may complicate bone marrow transplantation, in association with graft versus host disease.
Kidney diseases that affect tubules and interstitium, such as interstitial nephritis, will not cause nephrotic syndrome.
The above causes of nephrotic syndrome are largely those for adults, and this article will concentrate primarily on
adult nephrotic syndrome. However, nephrotic syndrome in infancy and childhood is an important entity. For
discussion of this topic, see the Medscape Reference article Pediatric Nephrotic Syndrome.
From a therapeutic perspective, nephrotic syndrome may be classified as steroid sensitive, steroid resistant, steroid
dependent, or frequently relapsing.
Corticosteroids (prednisone), cyclophosphamide, and cyclosporine are used to induce remission in nephrotic
syndrome. Diuretics are used to reduce edema. Angiotensin-converting enzyme (ACE) inhibitors and angiotensin II
receptor blockers are administered to reduce proteinuria. (See Treatment and Medication.)

Contributor Information and Disclosures

Author
Eric P Cohen, MD Professor, Department of Medicine, Division of Nephrology, Medical College of Wisconsin;
Nephrology Section Chief, Zablocki Veterans Affairs Hospital
Eric P Cohen, MD is a member of the following medical societies: American Society of Nephrology, Central
Society for Clinical Research, International Society of Nephrology, and Radiation Research Society
Disclosure: Nothing to disclose.
Coauthor(s)
Ramapriya Sinnakirouchenan, MD Fellow, Division of Nephrology, Medical College of Wisconsin
Ramapriya Sinnakirouchenan, MD is a member of the following medical societies: American Society of
Nephrology and Indian Medical Association
Disclosure: Nothing to disclose.
Specialty Editor Board
Laura Lyngby Mulloy, DO, FACP Professor of Medicine, Chief, Section of Nephrology, Hypertension, and
Transplantation Medicine, Glover/Mealing Eminent Scholar Chair in Immunology, Medical College of Georgia
Disclosure: Nothing to disclose.
Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center
College of Pharmacy; Editor-in-Chief, Medscape Drug Reference
Disclosure: Medscape Salary Employment
Eleanor Lederer, MD Professor of Medicine, Chief, Nephrology Division, Director, Nephrology Training
Program, Director, Metabolic Stone Clinic, Kidney Disease Program, University of Louisville School of Medicine;
Consulting Staff, Louisville Veterans Affairs Hospital
Eleanor Lederer, MD is a member of the following medical societies: American Association for the Advancement
of Science, American Federation for Medical Research, American Society for Biochemistry and Molecular
Biology, American Society for Bone and Mineral Research, American Society of Nephrology, American Society
of Transplantation, International Society of Nephrology, Kentucky Medical Association, National Kidney
Foundation, and Phi Beta Kappa
Disclosure: Dept of Veterans Affairs Grant/research funds Research; American Society of Nephrology Salary

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ASN Council Position; University of Louisville Salary Employment

Chief Editor
Vecihi Batuman, MD, FACP, FASN Huberwald Professor of Medicine, Section of Nephrology-Hypertension,
Tulane University School of Medicine; Chief, Renal Section, Southeast Louisiana Veterans Health Care System
Vecihi Batuman, MD, FACP, FASN, is a member of the following medical societies: American College of
Physicians, American Society of Hypertension, American Society of Nephrology, and International Society of
Nephrology
Disclosure: Nothing to disclose.

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