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Key Features

  • Clinical constellation of glomerulonephritis and pulmonary hemorrhage

  • Injury mediated by antibodies to epitopes in glomerular basement membrane (GBM)

  • Up to one-third of patients with anti-GBM glomerulonephritis have no evidence of concomitant lung injury

  • 10–20% of patients with rapidly progressive acute glomerulonephritis have circulating anti-GBM antibodies

  • Incidence peaks in the second and third decades 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 predominance

  • Lung involvement has been associated with

    • Pulmonary infection

    • Tobacco use

    • Hydrocarbon solvent exposure

    • Presence of HLA-DR2 and -B7 antigens

Clinical Findings

  • 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 renal dysfunction)

Diagnosis

  • 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

    • Light microscopy typically shows crescent formation

    • Immunofluorescence shows linear IgG staining along the GBM

Treatment

  • 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

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