From the Guidelines
Nephrotic syndrome is primarily caused by glomerular diseases, including minimal change disease, focal segmental glomerulosclerosis (FSGS), and membranous nephropathy.
Causes of Nephrotic Syndrome
- Primary glomerular disease: The most common causes of nephrotic syndrome, including minimal change disease, FSGS, and membranous nephropathy 1.
- Secondary glomerular disease: Caused by systemic autoimmune diseases, infections, drugs, or malignancy 1.
- Genetic defects: Congenital nephrotic syndrome can be caused by genetic defects in podocytes, such as NPHS1, NPHS2, WT1, and PLCE1 1.
- Infections: Certain infections, such as congenital infections or maternal allo-immune disease, can cause congenital nephrotic syndrome 1.
Key Points
- Nephrotic syndrome is characterized by heavy proteinuria, hypoalbuminemia, and edema 1.
- The prevalence of nephrotic syndrome is estimated at 2 to 7 per 100,000 children worldwide 1.
- Black children with nephrotic syndrome have relatively higher steroid resistance rates than children of other ethnicities 1.
- FSGS is observed more frequently in Afro-Caribbean patients than in Caucasians, with prevalence as high as 80% among nephrotic patients 1.
From the Research
Causes of Nephrotic Syndrome
The causes of nephrotic syndrome can be primary or secondary. Primary causes include:
- Minimal change disease (MCD), which is the most common primary cause of nephrotic syndrome in children 2, 3
- Focal segmental glomerulosclerosis (FSGS), which is the second most common primary cause of nephrotic syndrome in children and the most common primary cause in adults of African ancestry 2, 4, 3
- Membranous nephropathy (MN), which is the most common primary cause of nephrotic syndrome in white adults 4, 3
Secondary causes include:
- Diabetes mellitus, which is the most common secondary cause of nephrotic syndrome in adults 4, 3, 5
- Systemic lupus erythematosus 4, 3
- Amyloidosis 4, 3
- Infections 2, 4, 3
- Drugs 2
- Hematologic malignancies 3
Genetic Basis
Nephrotic syndrome can also be caused by genetic mutations, particularly in genes involved in the structure and function of the glomerular filtration barrier, such as nephrin and other podocyte genes 6.
Disease Processes
Nephrotic syndrome encompasses a variety of disease processes leading to heavy proteinuria and edema, and its progression and prognosis are variable and depend on etiology 2.