Goodpasture’s syndrome

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

Definition
Goodpasture’s syndrome is a form of rapidly progressive glomerulonephritis, which involves a progressive decrease in the kidney’s ability to function properly, accompanied by a cough with bloody sputum.

Causes, incidence, and risk factors

In Goodpasture’s syndrome, antibodies collect in both the kidney glomerulus and the tiny air sacs (alveoli) in the lungs, causing both glomerulonephritis and bleeding in the lungs.

The exact cause is unknown. It is an autoimmune disorder, which means that immune cells attack the body’s own organs, causing inflammation. Sometimes the disorder is triggered by a viral respiratory infection or by inhalation of hydrocarbon solvents. In such cases, the immune system may attack organs or tissues because it mistakes them for these viruses or foreign chemicals.

There is an inherited predisposition for Goodpasture’s syndrome and researchers believe that the gene or genes involved affect the way the immune system reacts to certain invaders, increasing the likelihood mistaken identity in the lungs and kidneys.

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. Other early symptoms include protein and blood in the urine. The condition progresses rapidly to kidney failure. 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

While listening with a stethoscope during a physical examination, the doctor may notice signs of fluid buildup due to bleeding in the lungs. Blood pressure may be high. The body may develop swelling as kidney function deteriorates. A 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-like deposits indicating rapidly progressive glomerulonephritis.

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. Other immune suppressants, such as cyclophosphamide, are used aggressively to try to stop the attacks by immune cells on the kidneys and lungs.

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

Expectations (prognosis)
The outcome varies. Early diagnosis and treatment tend to lead to better a outcome.

Complications

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

Call your health care provider if the amount of urine you produce drops.

Prevention
Avoid glue sniffing and siphoning gasoline, which expose the lungs to hydrocarbon solvents and can cause the disease in some cases. Early diagnosis and treatment may slow the progression of the disorder.

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