GOODPASTURE SYNDROME

Goodpasture's Syndrome: Description: Goodpasture's[MIM*233450] glomerulonephritis of the anti-basement membrane type associated with or preceded by hemoptysis; the nephritis usually progresses rapidly to produce death from renal failure, and the lungs at autopsy show extensive hemosiderosis or recent hemorrhage. Alternative Names: Anti-glomerular basement membrane antibody disease; Rapidly progressive glomerulonephritis with pulmonary hemorrhage; Pulmonary renal syndrome; Glomerulonephritis - pulmonary hemorrhage. Goodpasture's disease is an uncommon condition which can cause rapid destruction of the kidneys and bleeding into the lungs. It was first described by Ernest Goodpasture in 1919, and subsequently his name was given to the disease. However it is now known that several diseases can cause a similar picture. Most physicians reserve the name Goodpasture's disease (or syndrome) for the disease produced when the immune system attacks a particular molecule that is found in the kidney and the lung, the Goodpasture antigen. Confusingly, some use the term 'Goodpasture Syndrome' to refer to any patient with lung hemorrhage and severe nephritis, regardless of which disease caused it. Goodpasture's syndrome is a rare disease that can affect the lungs and kidneys. It is an autoimmune disease — a condition in which the body's own defense system reacts against some part of the body itself. When the immune system is working normally, it creates antibodies to fight off germs. In Goodpasture's syndrome, the immune system makes antibodies that attack the lungs and kidneys. Why this happens is not fully understood. Researchers have identified a number of possible causes, among them the presence of an inherited component; exposure to certain chemicals, including hydrocarbon solvents and the weed killer Paraquat; and viral infections. Goodpasture's syndrome is rare. In European populations between half and one case presents per million people per year. It is less likely to be found in non-European populations. While cases have occurred in patients between the ages of 4 and 80, it is most common between ages 18 and 30 and again between 50 and 65. Males are six times more affected than females. Men are eight times more likely to be affected than women. The disease most commonly occurs in early adulthood. Symptoms: Symptoms include: Bloody urine.
  • Chest pain.
  • Cough with bloody sputum (coughing up blood).
  • Dark colored urine.
  • Decreased urine output.
  • Difficulty breathing after activity.
  • Foamy urine.
  • Nausea and vomiting.
  • Pale skin.
  • Weakness.
Causes and Risk factors: Goodpasture syndrome is an autoimmune disorder. This means your body makes antibodies that attack your own body tissues. In this case, antibodies form against a certain type of protein called collagen. The collagen is present in the alveoli (tiny air sacs in the lungs) and in the glomeruli (the filtering units of the kidney). These antibodies are called anti-glomerular basement membrane antibodies (or anti-GBM antibodies). Sometimes the disorder is triggered by a viral respiratory infection or by inhaling hydrocarbon solvents. In such cases, the immune system may attack organs or tissues because it mistakes them for these viruses or foreign chemicals. The antibody attack leads to bleeding in the air sacs, which causes shortness of breath, cough, and bloody sputum. It also causes inflammation in the glomeruli of the kidney, which causes blood in urine (hematuria), protein in the urine (proteinuria), or kidney failure. Diagnosis: During a physical examination, the health care provider will usually discover that the patient has high blood pressure. The patient usually has signs of fluid overload, such as swelling, gallop rhythms of the heart, and crackle sounds in the lungs. The crackles may also be from blood in the air sacs. The following are relevant test results: Urinalysis shows blood and protein in the urine. Abnormal red blood cells may be seen.
  • BUN and creatinine levels are elevated.
  • Chest x-ray shows diseased alveoli.
  • Arterial blood gas analysis may show low oxygen in the blood.
  • Lung biopsy shows damaged alveoli.
  • Kidney biopsy shows damaged glomeruli.
  • Anti-GBM antibody levels are elevated.
If Goodpasture syndrome is not diagnosed and treated right away, the damage from the disease can lead to end-stage renal failure, in which the kidney ceases to function. Being exposed to hydrocarbons, smoking, and having a lung infection may increase a person's risk of bleeding from lungs with Goodpasture syndrome. Treatment: Goodpasture's syndrome is treated with oral immunosuppressive drugs-cyclophosphamide and corticosteroids-to keep the immune system from making antibodies. Corticosteroid drugs may be given intravenously to control bleeding in the lungs. A process called plasmapheresis may be helpful and necessary to remove the harmful antibodies from the blood. In plasmapheresis, a patient's blood is drawn, about 300 ml at a time, and placed in a centrifuge to separate the red and white blood cells from the plasma. The cells are then placed in a plasma substitute and returned to the body. This procedure is usually done in combination with immunosuppressive drug treatment. Goodpasture's syndrome may last only a few weeks or as long as 2 years. Bleeding in the lungs can be very serious and even fatal in some cases. But Goodpasture's syndrome does not usually lead to permanent lung damage. Damage to the kidneys, however, may be long-lasting. If the kidneys fail, dialysis to remove waste products and extra fluid from the blood, or kidney transplantation, may become necessary. Medicine and medications: Prednisone (Deltasone, Orasone, Meticorten, Sterapred).
  • Methylprednisolone (Solu-Medrol).
  • Cyclophosphamide (Cytoxan).
  • Azathioprine (Imuran).
  • Trimethoprim and sulfamethoxazole (Septra, Bactrim DS).
Note: The following drugs and medications are in some way related to, or used in the treatment. This service should be used as a supplement to, and NOT a substitute for, the expertise, skill, knowledge and judgment of healthcare practitioners. DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.  

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