Nephrotic syndrome classification

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Yazan Daaboul, Serge Korjian

Overview

Nephrotic syndrome can be classified into primary or secondary depending 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

A broad classification of nephrotic syndrome based on 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 are:

• Minimal change disease
• Focal segmental glomerulosclerosis (FSGS)
• Membranous nephropathy
• Membranoproliferative glomerulonephritis
• IgM nephropathy
• C1q nephropathy

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}