Goodpasture’s syndrome

Goodpasture’s syndrome

Definition:

A form of rapidly progressive glomerulonephritis (inflammation of the kidney glomeruli) involving progressive decrease in kidney function, accompanied by a cough with bloody sputum.

Alternative Names:

Anti-glomerular basement membrane antibody disease; Rapidly progressive glomerulonephritis with pulmonary hemorrhage; Pulmonary renal syndrome; Glomerulonephritis - pulmonary hemorrhage

Causes, incidence, and risk factors:

The disorder is characterized by deposits of antibodies in the basement membranes of both the kidney glomerulus and the lung alveoli, causing both glomerulonephritis and pulmonary (lung) bleeding.

The exact cause is unknown. It is an autoimmune disorder (action of the immune system against normal body tissues). Sometimes the disorder is triggered by a viral infection or by inhalation of hydrocarbon solvents.

It may develop after a recent viral respiratory infection. There is an inherited predisposition for Goodpasture's syndrome.

The disorder usually manifests with excretion of protein and blood in the urine. It progresses rapidly to kidney failure. Antibody deposits in the lungs cause bleeding within the lung tissues, resulting in the bloody sputum, which may be one of the first symptoms of the disorder. Iron deficiency anemia may be present, as well as anemia associated with renal failure, and may be worse than expected considering the amount of kidney damage.

Symptoms:

Bloody urine
Dark colored urine
Decreased urine output
Foamy urine
Cough with bloody sputum (coughing up blood)
Difficulty breathing after exertion
Weakness
Nausea/vomiting
Nonspecific chest pain
Pale skin

Signs and tests:

Auscultation of the lungs may indicate fluid (from bleeding in the lungs). Blood pressure may be high. Edema (swelling) of the body may develop as kidney function deteriorates. Skin rash may be observed in some cases.

A CBC often indicates anemia.
Serum iron and ferritin may be low.
BUN and creatinine levels increase as kidney function decreases.
A urinalysis may show protein, blood, casts or other abnormality.
Serum antibody to normal human glomerular basement membrane is positive.
Sputum stain may indicate macrophages (immune system cells that respond to the presence of antibodies) that contain iron pigments.
A chest X-ray shows fluid in the lung tissues.
A lung needle biopsy shows immune system deposits.
A kidney biopsy shows immune system deposits, with crescent- deposits indicating rapidly progressive glomerulonephritis.

Treatment:

Treatment is focused on slowing progression of the disease. It is most effective when begun early, before kidney function has deteriorated to the point of requiring dialysis. Corticosteroids or other anti-inflammatory agents may be used to reduce the immune response, with variable results. Immune suppressants such as cyclophosphamide are used aggressively to reduce immune system effects.

Plasmapheresis, a procedure by which blood plasma is removed from the body and replaced with fluids or donated plasma, may be performed daily for 2 or more weeks to remove circulating antibodies. It is fairly effective in slowing or reversing the disorder.

Dialysis may be required if kidney function is poor.

Kidney transplant may be quite successful, especially if performed after circulating antibodies have been absent for several months.

Expectations (prognosis):

The probable outcome is variable. Most cases progress to severe renal failure and end-stage renal disease within months. Early diagnosis and treatment makes the prognosis (probable outcome) more favorable.

Complications:

Rapidly progressive glomerulonephritis
Chronic renal failure
End-stage renal disease
Severe pulmonary hemorrhage (lung bleeding)
Respiratory failure

Calling your health care provider:

Call for an appointment with your health care provider if symptoms indicating Goodpasture's syndrome are present.

Call your health care provider if urine output decreases.

Prevention:

There is no known prevention for Goodpasture's syndrome. Avoid glue sniffing and siphoning gasoline. Early diagnosis and treatment may slow the progression of the disorder.

Review Date: 2/5/2001
Reviewed By: David A. Kaufman, M.D., Pulmonary and Critical Care Medicine, Hospital of the University of Pennsylvania, Philadelphia, PA. Review provided by VeriMed Healthcare Network.

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