Anti-GBM disease or glomerular basement membrane antibody disease is also known as Goodpasture's syndrome when there is concomittant lung involvement. It is rare, about or less than one in a million. It is a renal-pulmonary syndrome or just renal meaning it can affect lungs and kidneys. When it affects the lungs it causes pulmonary hemorrhage. In the kidneys it causes glomerulonephritis.
Anti-glomerular basement membrane (GBM) antibody GN is a rare glomerular disease with an incidence of 0.5–1 per million population. It is caused by autoantibodies against the noncollagenous domain of the a3 chain of type IV collagen. Anti-GBM GN may present either as an isolated kidney disease or as a pulmonary–renal syndrome (Goodpasture’s syndrome). Anti-GBM is usually a rapidly progressive crescentic GN, and about 80% of patients have crescents in half or more of their glomeruli. Goodpasture’s syndrome occurs in 40%–60% of patients, and kidney disease is accompanied by sometimes massive and fatal pulmonary hemorrhage. AntiGBM disease with pulmonary involvement is more frequent in men (about 80%) and typically occurs during the second decade. Isolated anti-GBM nephritis does not have clear male preponderance and may also occur in older persons. If untreated, anti-GBM disease has very high morbidity, with almost all patients going on to kidney failure, and it can have significant mortality. In patients with Goodpasture’s syndrome, the mortality rate was 96% before the introduction of immunosuppression, and 47% despite treatment with immunosuppression. Most patients died of respiratory failure. The cornerstone of the treatment is rapid removal of the pathogenic autoantibodies and suppression of their production to prevent further kidney and pulmonary injury. [1]
GLomeruli are
This is mostly a male disease in those with Goodpasture's syndrome but in renal only disease, it is equally distributed among genders. Goodpasture's occurs in young men but nephritis only from GBM antibodies can occur at an older age. [1]
