Goodpasture syndrome, also known as anti-glomerular basement membrane (anti-GBM) disease, is a rare autoimmune disorder that primarily affects the kidneys and lungs. It is characterized by the presence of antibodies that target the glomerular basement membrane, leading to inflammation and damage in these organs.
The syndrome was first described by Ernest Goodpasture in 1919, hence its name. It typically presents with symptoms such as cough, shortness of breath, fatigue, and hemoptysis (coughing up blood). Kidney involvement may manifest as blood in the urine, proteinuria (excessive protein in the urine), and impaired kidney function.
Synonyms for Goodpasture syndrome include anti-GBM disease, Goodpasture's disease, and anti-glomerular basement membrane antibody disease. These terms are often used interchangeably to refer to the same condition.
Diagnosis of Goodpasture syndrome involves various tests, including blood tests to detect the presence of anti-GBM antibodies, imaging studies to assess lung and kidney involvement, and kidney biopsy to confirm the presence of glomerular basement membrane damage.
Treatment for Goodpasture syndrome typically involves a combination of immunosuppressive medications, such as corticosteroids and cyclophosphamide, to suppress the immune response and reduce inflammation. Plasma exchange therapy may also be employed to remove the circulating antibodies from the blood.
Early diagnosis and prompt treatment are crucial in managing Goodpasture syndrome and preventing further organ damage. Without intervention, the disease can progress rapidly and lead to severe kidney failure and respiratory failure.
Regular follow-up with healthcare professionals is essential for individuals with Goodpasture syndrome to monitor kidney and lung function, adjust medications, and manage potential complications.