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

Below table lists different types of nephrotic syndromes:

Different types of nephrotic syndromes	Disease name
Podocytopathies	Primary	Primary minimal change disease
		Primary FSGS
	Secondary	Infection: HIV
		Drugs/toxins: NSAIDs, interferon, pamidronate, lithium, vaccins, envenomation
		Malignancies: hodgkin lymphoma, thymoma
		Genetics: nephrin mutations, podocin
Membranous glomerulonephritis	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	Alport syndrome Fabry disease Nail-patella syndrome Partial lipodystrophy Preeclampsia

• 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.