Nephrotic syndrome classification

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Ali Poyan Mehr, M.D. [2]; Associate Editor(s)-in-Chief: Olufunmilola Olubukola M.D.[3] Yazan Daaboul, Serge Korjian

Overview

Nephrotic syndrome can be classified into primary or secondary depending on the underlying etiology. Primary (idiopathic) nephrotic syndrome is defined as nephrotic syndrome due to a primary glomerular disease. Secondary nephrotic syndrome is defined as nephrotic syndrome due to a primary etiology other than glomerular disorders, such as infections, malignancies, systemic conditions, and medications.

Classification

Etiologic Classification

Nephrotic syndrome can be classified into primary or secondary depending on the underlying etiology.

Nephrotic syndrome

Primary

Secondary

Primary (Idiopathic) Nephrotic Syndrome

Primary (idiopathic) nephrotic syndrome is defined as nephrotic syndrome due to a primary glomerular disease. It is a common diagnosis in children. Etiologies of idiopathic nephrotic syndrome include:

Different types of nephrotic syndromes		Disease name
Podocytopathies	Primary	Primary minimal change disease
		Primary FSGS
	Secondary	Infection: HIV
		Drugs/toxins: NSAIDs, interferon, pamironate, lithium, vaccins, envenomation
		Malignancies: hodgkin lymphoma, thymoma
		Genetics: nephrin mutations, podocin
Membranous nephropathies	Primary	Primary membranous nephropathy
	Secondary	Drugs, toxins, malignancies
Membranoproliferative glomerulonephritis	Secondary	Autoimmune disorders: SLE, aquired C3 glomerulopathy Infections: HCV, endocarditis Genetics Malignancies Thrombotic microangiopathy
Glomerular deposition diseases		Diabetes mellitus Amyloidosis Monoclonal deposition disease Nodular glomeruloneohritis associated with heavy smoking
Other lesions

• Minimal change disease
• Focal segmental glomerulosclerosis (FSGS)
• Membranous nephropathy
• Diabetic nephropathy
• Glomerular deposition diseases
  • Light chain deposition disease
  • Renal amyloidosis
  • Fibrillary-Immunotactoid Glomerulopathy
  • Fabry's Disease
• Hereditary Nephropathies: are group of disorders that present with both the features of nephrotic and nephritic syndromes. When features of nephritic syndrome are associated with sensorineural deafness, it is called Alport’s syndrome. Other hereditary nephropathies include IgA Nephropathy, C1q Nephropathy, etc.

Secondary Nephrotic Syndrome

Secondary nephrotic syndrome is defined as nephrotic syndrome due to a primary etiology other than glomerular disorders, such as infections, malignancies, systemic conditions, and medications. The most common cause of secondary nephrotic syndrome is diabetes mellitus.

Common Causes of Secondary Nephrotic Syndrome

Cause	Characteristic Features
Diabetes Mellitus	Glucosuria Hyperglycemia Polyuria Polydipsia
Systemic Lupus Erythematosus (SLE)	Anemia Arthralgia Malar or discoid rash Photosensitivity Neurological, renal, immunologic disease Pleural effusion or ascites Positive ANA and anti-dsDNA antibodies
Viral Hepatitis (HBV and HCV)	Elevated liver function tests History of transfusions High-risk sexual encounters IV drug use Positive HBsAg or HCV RNA
NSAIDs	Associated with minimal change disease (MCD)
Amyloidosis	Cardiomyopathy Hepatomegaly Peripheral neuropathy
Multiple Myeloma	Abrnomal urine protein electrophoresis (UPEP) and serum protein electrophoresis (SPEP) Back pain Renal failure Anemia Hypercalcemia Positive serum beta-2-microglobulin
HIV	Reduced CD4 cell counts High-risk sexual encounters History of transfusions History of drug use Renal pathology similar to focal segmental glomerulosclerosis (FSGS)
Preeclampsia	High blood pressure, edema, and proteinuria during pregnancy

^{Adapted from Hull RP, Goldsmith DJ. Nephrotic syndrome in adults. BMJ. 2008; 336:1185-9}