Primary Glomerular Disorders That Can Cause Nephrotic Syndrome

Congenital and infantile nephrotic syndrome

These rare disorders are inherited. Congenital nephrotic syndrome (Finnish type) and diffuse mesangial sclerosis are the 2 main causes. They closely resemble focal segmental glomerulosclerosis. Symptoms are present at birth in the Finnish type and develop during childhood in diffuse mesangial sclerosis.

These disorders do not respond to corticosteroids. Because the level of albumin in the blood is extremely low, removal of both kidneys is often considered. Supportive therapy, including dialysis, is given until the child is eligible for a kidney transplant.

Focal segmental glomerulosclerosis

This disease damages glomeruli. It affects mainly adolescents but also young and middle-aged adults. It is more common in Black men.

The prognosis is poor because treatment is not very effective. The disease progresses to kidney failure within 15 years in two-thirds of people.

Membranoproliferative glomerulonephritis

This uncommon type of glomerulonephritis (which can cause nephrotic syndrome despite its name) can occur in children with no obvious cause, or in adults, usually with an identifiable cause. It is caused by deposits of immune complexes (combinations of antigens and antibodies) attaching to the kidneys, sometimes for unknown reasons. Antibodies are proteins made by the body to attack specific molecules called antigens.

If caused by a disorder that can be treated (for example, systemic lupus erythematosus or hepatitis B or C infection), a partial remission may occur. The outcome is not as good in people in whom the cause remains unknown.

Membranous nephropathy

This serious type of glomerular disease affects mainly adults.

In about one-third of people, the disease goes away on its own; about one-third develop kidney failure. The rest continue to have protein in the urine, as either the nephrotic syndrome or the asymptomatic proteinuria and hematuria syndrome.

Mesangial proliferative glomerulonephritis

This disorder accounts for about 3 to 5% of people with nephrotic syndrome of unknown cause. It affects people of all ages.

Approximately 50% of people initially respond to corticosteroids. About 10 to 30% develop progressive kidney failure. Relapses may respond to cyclophosphamide.Approximately 50% of people initially respond to corticosteroids. About 10 to 30% develop progressive kidney failure. Relapses may respond to cyclophosphamide.

Minimal change disease

This mild disease of the glomerulus is more common in children but also affects adults.

The prognosis is good, and more than 80% of people achieve remission after treatment.