Goodpasture’s syndrome - what is and definition

An autoimmune disorder in which the IMMUNE SYSTEM produces antibodies that attack the glomeruli in the KIDNEYS, impairing kidney function, and the alveoli in the LUNGS, causing bleeding into the lung tissue. In most people who develop Goodpasture’s syndrome the symptoms follow a viral INFECTION of the upper respiratory tract or exposure to environmental toxins, notably hydrocarbons. Because Goodpasture’s syndrome tends to run in families, researchers believe a GENE MUTATION is likely responsible.

Siphoning gasoline and sniffing aerosols such as paints and glues are the most common exposures to hydrocarbons that can result in Goodpasture’s syndrome.

Though the coughing up of bloody SPUTUM (HEMOPTYSIS) is the first and often the more distressing sign of Goodpasture’s syndrome, GLOMERULONEPHRITIS is the more serious consequence, leading rapidly in many people to RENAL FAILURE. ANEMIA (insufficient erythrocytes in the blood) and HYPERTENSION (elevated BLOOD PRESSURE), consequences of the renal failure, may quickly become significant.

The diagnostic path includes blood and urine tests to assess kidney function, chest X-RAY to detect accumulated fluid in the lungs, and biopsy of lung and kidney tissue to confirm the presence of antibodies. The course of Goodpasture’s syndrome may run two months to several years. Early diagnosis allows aggressive interventions, including plasmapheresis to remove antibodies from the bloodstream and IMMUNOSUPPRESSIVE THERAPY to prevent the immune system from producing further antibodies. These interventions can mediate the syndrome’s progression, minimizing damage to the kidneys. Though about 90 percent of those who develop this once-fatal syndrome now survive, many of them continue to experience progressive renal failure that results in END-STAGE RENAL DISEASE (ESRD).

See also ALVEOLUS; ANTIBODY; AUTOIMMUNE DISORDERS; HEMAPHERESIS; GLOMERULUS; RENIN.

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