Dr. Goodpasture first described this syndrome in 1919 during the influenza epidemic. Goodpasture’s syndrome is also called anti-glomerular basement (GBM) antibody disease because of the glomerular basement membrane autoantibodies that attack the lungs and kidneys in this condition. The kidney condition in this disorder is known as glomerulonephritis, which refers to inflammation in the kidney’s functional units, the glomeruli. About 60-80 percent of patients have both lung and kidney involvement. 20-40 percent have kidney involvement alone, and less than 10 percent of patients only have lung involvement.
Who Is Affected?
Goodpasture’s syndrome is a rare disorder most likely to affect children and young adults although individuals of any age may be affected. Males are affected 8 times more often than females. White people are affected more often than blacks, and certain ethnic groups may be more susceptible, such as the Maoris of New Zealand.
Young men ages 20-30 years are more likely to present with a pulmonary-renal syndrome, and elderly women ages 60-70 years present primarily with glomerulonephritis alone. Approximately 1-2 percent of all cases of rapidly progressive glomerulonephritis are secondary to Goodpasture's syndrome.
Symptoms and Disease Course
Like most autoimmune diseases, the disease course and symptoms in Goodpasture’s can vary in severity. Some patients develop renal (kidney) failure and severe pulmonary hemorrhages leading to asphyxia or difficulty in breathing. Other patients may only suffer small amounts of bleeding, either from coughing up blood or episodes of blood in their urine.
Typically in the early stages of this disease, symptoms are vague and include fatigue, chest pain, nausea, chills, fever, weight loss, difficult breathing, arthralgia, and anemia. Some individuals cough up blood or notice a burning sensation when urinating. A urine exam on a routine physical may show blood and/or protein. Patients may also exhibit hypertension, enlarged liver and spleen, cyanosis, and skin rash.
Goodpasture’s syndrome may last for a few weeks and resolve spontaneously or it may persist for as long as 2 years. Pulmonary bleeding can be very serious and sometimes fatal. Fortunately, Goodpasture’s does not usually lead to permanent lung damage. However, damage to the kidneys is often rapidly progressive and long lasting.
Autoimmunity and Genetics
GBM antibodies were first discovered in 1967. Their presence in the blood circulation of patients with diffuse pulmonary hemorrhage and glomerulonephritis demonstrated the autoimmune nature of this disorder.
In Goodpasture’s syndrome, GBM antibodies cause tissue injury by binding to cellular receptors in the basement membranes. A layer of immunoglobulins (antibodies) can be demonstrated along the glomerular basement membrane, and in some cases, the alveolar basement membrane of the lungs.
The basement layer of these organs is composed primarily of type IV collagen, which acts as a support structure for the tissues. While the lungs are typically blocked from this antibody attack, various factors allow antibody invasion. These factors include non-specific lung injuries, tobacco smoking, upper respiratory bacterial and viral infections, high concentrations of inspired oxygen, endotoxins, bacteremia, inhaled cocaine, and exposure to metal dusts, volatile hydrocarbons and organic solvents.
An increased incidence of Goodpasture’s syndrome occurs in people with certain immune system antigens, including HLA-DR2 and HLA-DRW15. Also, HLA-B7 is found more frequently and is associated with more severe cases of kidney disease.
Diagnosis
Urinalysis usually shows abnormal concentrations of blood, protein, and red blood cells. Blood tests for anti-GBM antibodies are elevated. Blood tests for BUN and creatinine are elevated and indicate the severity of the kidney disease. Arterial blood gas measurements and pulmonary function tests are also used to evaluate pulmonary function. A lung biopsy and kidney biopsy may be performed to assess the extent of the tissue damage.
Treatment
The main goal of treatment is to remove anti-GBM antibodies from the blood circulation. This is accomplished through plasmapheresis. Prednisone and other cytotoxic medications such as cyclophosphamide may also be used. If kidney failure exists, dialysis may be needed. If antibody titers fall and kidney function remains impaired, a kidney transplant may be necessary.
Resources:
Sat Sharma, Goodpasture Syndrome, eMedicine, Jan 2007, accessed Sept 5, 2009.
David Keren and Jeffrey Warren, Diagnostic Immunology, Baltimore, MD, William & Wilkins, 1993.
Elaine Moore - I'm a retired medical technologist and medical writer with more than 30 years experience working in hospital laboratories. Currently, I ...
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