Rapidly progressive glomerulonephritis

Definition

Rapidly progressive glomerulonephritis is a form of kidney disease that causes damage to the small structures (glomeruli) inside the kidneys that help filter waste and fluids from blood to form urine. The disease leads to a rapid loss of kidney function.

Alternative Names

Necrotizing glomerulonephritis; Glomerulonephritis - crescentic; Crescentic glomerulonephritis

Causes

Many conditions are known to cause or increase the risk for developing rapidly progressive glomerulonephritis. These include:

• Abscess of any internal organ
• Anti-glomerular basement membrane antibody disease
• Blood vessel diseases such as vasculitis or polyarteritis
• Collagen vascular disease such as lupus nephritis and Henoch-Schonlein purpura
• Goodpasture syndrome
• IgA nephropathy
• Membranoproliferative GN

The following increase your risk of developing this condition:

• History of cancer
• Blood or lymphatic system disorders
• Exposure to hydrocarbon solvents

Rapidly progressive glomerulonephritis includes any type of glomerulonephritis (inflammation of the glomerulus) in which progressive loss of kidney function occurs over weeks to months.

The disorder is more common in certain geographic areas. Mini-epidemics of this disorder have also occurred. Rapidly progressive glomerulonephritis is most common in people age 40-60, and slightly more common in men. It is unusual in preschool children, and slightly more common in later childhood.

Symptoms

Common symptoms include the following:

• Edema (swelling) of the face, eyes, ankles, feet, legs, or abdomen
• Blood in the urine
• Dark or smoke-colored urine
• Decreased urine volume

Symptoms that may also appear include the following:

• Abdominal pain
• Cough
• Diarrhea
• General ill feeling
• Fever
• Joint aches
• Muscle aches
• Loss of appetite
• Shortness of breath

Exams and Tests

A physical examination reveals edema (swelling). The doctor will listen to your heart and lungs with a stethoscope. Abnormal heart and lung sounds may be present. Blood pressure may be high.

Rapid, worsening loss of kidney function may be present. This disease may show up as an acute nephritic syndrome or unexplained kidney failure.

Tests that may be done include:

• Anti-glomerular basement membrane antibody tests
• Antineutrophil cytoplasmic antibodies (ANCAs)
• BUN and creatinine
• Complement levels
• Creatinine clearance
• Urinalysis

Other tests for suspected causes may be done. A kidney biopsy confirms the diagnosis. Most pathologists define crescentic glomerulonephritis when 50% or more glomeruli have an abnormal crescent shape on a kidney biopsy.

Treatment

Treatment depends on the underlying cause.

Corticosteroids may relieve symptoms in some cases. Medications that suppress the immune system may also be prescribed, depending on the cause.

A procedure called plasmapheresis may relieve the symptoms in some cases. The fluid part of the blood containing antibodies is removed and replaced with intravenous fluids or donated plasma (without antibodies). The removal of antibodies may reduce inflammation in the kidney tissues.

Persons with this condition should be closely watched for signs of progression to kidney failure. Dialysis or a kidney transplant may ultimately be necessary.

Outlook (Prognosis)

Without treatment, crescentic glomerulonephritis often worsens rapidly to kidney failure and end-stage kidney disease in 6 months or less, although a few cases may just go away on their own.

Those who receive treatment may recover some or rarely all of their original kidney function. The extent of recovery is related to the degree of kidney function at diagnosis and degree of crescent formation. The disorder may recur.

If the disease occurs in childhood, it is likely that kidney failure will eventually develop.

Possible Complications

• Congestive heart failure
• Pulmonary edema
• Hyperkalemia
• Acute renal failure
• Chronic renal failure
• End-stage renal disease

When to Contact a Medical Professional

Call your health care provider if symptoms indicate rapidly progressive glomerulonephritis may be present.

If you have this disorder, call if new symptoms develop, especially irregular heartbeat, difficulty breathing, increased edema, or decreased urine production.

Prevention

The prompt treatment of disorders that can cause rapidly progressive glomerulonephritis may prevent the development of this disease.