Merck Manual

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Primary Glomerular Disorders That Can Cause Nephrotic Syndrome

Primary Glomerular Disorders That Can Cause Nephrotic Syndrome

Glomerular Disorder

Description

Prognosis

Minimal change disease

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

The prognosis is good. About 90% of children and nearly as many adults respond to treatment. In 30 to 50% of adults, disease relapses. After treatment for 1 or 2 years, more than 80% of people have permanent remission.

Focal segmental glomerulosclerosis

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

The prognosis is poor because treatment is not very effective. In most adults and children, the disease progresses to end-stage kidney failure 5 to 20 years after diagnosis.

Membranous nephropathy

This serious type of glomerular disease affects mainly adults. It is more common in whites.

In about 25% of people, protein stops being excreted in the urine. About 25% develop end-stage kidney failure. The rest continue to have protein in the urine, as either the nephrotic syndrome or the asymptomatic proteinuria and hematuria syndrome.

Congenital and infantile nephrotic syndrome

These rare disorders are inherited. Congenital nephrotic syndrome (Finnish type) and diffuse mesangial sclerosis are the two 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.

Membranoproliferative glomerulonephritis

This uncommon type of glomerulonephritis occurs primarily between the ages of 8 and 30. It is caused by deposits of immune complexes (combinations of antigens and antibodies) attaching to the kidneys, sometimes for unknown reasons.

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. About half of untreated people progress to end-stage kidney failure within 10 years and 90% within 20 years.

Mesangial proliferative glomerulonephritis

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

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