Definition
Goodpasture`s Syndrome is an autoimmune condition that affects the kidneys and the lungs. In a normal person, antibodies are produced to fight against anything foreign like an infection. In an autoimmune disease, the body produces antibodies that fail to recognize parts of the body, mistake them to be ‘foreign’ and launch an attack against them.
It is is a rare condition affecting 1 in 1 million people each year. It is also known as anti-glomerular basement membrane disease (Anti-GBM disease), rapidly progressive glomerulonephritis with pulmonary hemorrhage, or pulmonary renal syndrome.
History
Ernest Goodpasture first described the disorder in 1919. He reported a case of pulmonary hemorrhage and glomerulonephritis during an influenza epidemic. In 1955, Parkin described 3 cases of lung hemorrhage and nephritis that occurred in the absence of arteritis. In 1958, Stanton and Tang reported a series of young men with pulmonary hemorrhage and glomerulonephritis, similar to Goodpasture’s original description.
In the 1950’s, Krakower and Greenspon identified GBM as the antigen. In 1967, Lerner, Glassock, and Dixon confirmed that the antibodies taken from the diseased kidneys produced nephritis in experimental animals. The discovery of anti-GBM antibodies led to the understanding of the pathogenesis of Goodpasture syndrome.
Epidemiology of Goodpasture`s Syndrome
Anti-glomerular basement membrane (anti-GBM) disease is quite rare, with an estimated incidence of 1 to 2 cases per million. It causes 1% to 2% of all cases of glomerulonephritis. In one series, anti-GBM antibodies were found in 15% of patients presenting with a pulmonary renal syndrome. There is a male predominance with cases divided approximately 60% male, 40% female. There are 2 age peaks: at 20 to 30 years and at 60 to 70 years. Approximately 83% of cases occur in white people.
Risk factors
Insults to the lungs are probably required to produce both the renal and pulmonary disease.
- There is a strong genetic linkage to HLA-DRB1
- Exposure to organic solvents or hydrocarbons.
- Smoking
- Infection – eg, influenza
- Heavy smoker who had taken to using crack cocaine.
- Exposure to metal dusts
- It can occur after renal transplantation in Alport’s syndrome
Activities that causes Goodpasture’s syndrome
Its precise cause is unknown, but an insult to the blood vessels taking blood from and to the lungs is believed to be required to allow the anti-GBM antibodies to come into contact with the alveoli. Examples of such an insult include:
- Exposure to organic solvents (e.g. chloroform) or hydrocarbons
- Exposure to tobacco smoke
- Certain gene mutations (HLA-DR15)
- Infection, such as influenza A
- Cocaine inhalation
- Metal dust inhalation
- Bacteraemia
- Sepsis
- High-oxygen environments
- Treatment with antilymphocytic treatment (especially monoclonal antibodies)
Symptoms
- Fatigue
- Nausea and vomiting
- Difficulty breathing
- Pale skin
When Goodpasture syndrome affects the kidneys, symptoms may include:
- Blood in the urine
- Foamy urine
- Swelling in the legs
- High blood pressure
- Burning or difficulty when urinating
- Back pain below the ribs
- Swelling of the hands and feet
Diagnosis and Tests
He or she may order the following diagnostic tests:
- Urinalysis: High levels of protein and high numbers of red blood cells in urine may indicate kidney damage
- Blood test: A blood sample can be analyzed for the presence of antibodies that attack the lungs and kidneys
- Chest X-ray: Results can identify lung damage. For example, abnormal white patches are associated with lung bleeding
- Biopsy: A small sample of kidney or lung tissue may need to be removed to check for the presence of Goodpasture syndrome antibodies. An analysis of kidney tissue can also identify the extent of any kidney damage.
Treatment and Medication
- Treatment usually includes oral immunosuppressive drugs such as cyclophosphamide and corticosteroids. These drugs decrease the immune system’s production of Goodpasture syndrome antibodies.
- In some cases, intravenous corticosteroids may be needed to control bleeding in the lungs.
- Depending on the patient’s response to therapy, treatment with immunosuppressive drugs may continue for six to 12 months.
- Treatment of Goodpasture syndrome also usually includes a procedure called This helps remove harmful antibodies from the blood. During this procedure, about 300 milliliters of blood at a time are drawn from the body and placed in a centrifuge. The centrifuge separates the red and white blood cells from the plasma, the component that contains Goodpasture syndrome antibodies. Then the red and white blood cells are mixed into a plasma substitute and returned to the body. Usually, plasmapheresis is continued daily for several weeks.
- Until the disease runs its course, some patients may need to be placed on supplemental oxygen or a ventilator. Other patients may require blood transfusions.
Prevention of Goodpasture`s Syndrome
- Smoking cessation and avoidance of hydrocarbon exposure are important.
- If symptoms recur, a physician should be notified immediately. To prevent progression of this underlying kidney disease, BP should be controlled to 120/80 mmHg with blockers of the renin angiotensin system and hyperlipidaemia should be controlled to a goal of LDL <1.8 mmol/L (70 mg/dL).