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Clinical constellation of glomerulonephritis and pulmonary hemorrhage (Goodpasture syndrome)
Injury mediated by antibodies to epitopes in GBM
10–20% of patients with rapidly progressive acute glomerulonephritis have circulating anti-GBM antibodies
Incidence peaks in the third decade of life, during which time males are predominantly affected and lung involvement is more common, and again in the sixth and seventh decades with less gender specificity and pulmonary involvement
Lung involvement has been associated with
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Onset of disease may be preceded by an upper respiratory tract infection
Hemoptysis, dyspnea, and possible respiratory failure
Other findings are consistent with a rapidly progressive glomerulonephritis
Some cases may present with much milder forms of the nephritic spectrum of disease (eg, glomerular hematuria and proteinuria with minimal kidney dysfunction)
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Chest radiographs may demonstrate pulmonary infiltrates if pulmonary hemorrhage is present
Complement levels are normal
Circulating anti-GBM antibodies positive in > 90%
A minority of patients also have elevated ANCA titers; these patients should be treated with plasma exchange as for anti-GBM disease
Kidney biopsy
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Patients with pulmonary hemorrhage and strong clinical suspicion of Goodpasture syndrome should be treated emergently—possibly prior to confirming the diagnosis with serology and kidney biopsy
Combination of plasma exchange therapy daily for up to 2 weeks to remove circulating antibodies, and administration of corticosteroids and cyclophosphamide to prevent formation of new antibodies and control the inflammatory response
Rituximab has been used in a small number of patients with refractory disease
Long-term prognosis is poor in patients with oliguric AKI or requiring dialysis upon presentation