Guidelines
Guidelines Summary
The KDIGO 2021 Clinical Practice Guideline for the Management of Glomerular Diseases includes the following recommendations for the diagnosis and treatment of anti-GBM disease [42] :
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In all patients with suspected rapidly progressive glomerulonephritis (RPGN), serologic testing for the presence of anti-GBM antibodies should be done urgently using commercially available enzyme-linked immunoassays. In patients with negative results, kidney biopsy is required for diagnosis of anti-GBM disease.
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Treatment for suspected anti-GBM should be initiated before the diagnosis is confirmed
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Treat anti-GBM disease with cyclophosphamide and glucocorticoids plus plasmapheresis except those who are on dialysis at presentation, have 100% crescents or >50% global glomerulosclerosis in an adequate biopsy sample, and do not have pulmonary hemorrhage.
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Plasma exchange should be performed until anti-GBM titers are no longer detectable.
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Cyclophosphamide should be administered for 2–3 months and glucocorticoids for about 6 months
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No maintenance therapy of anti-GBM disease is necessary; patients who are anti-GBM- and ANCA-positive should be treated with maintenance therapy as for patients with ANCA-associated vasculitis (AAV)
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Consider rituximab for refractory anit-GBM disease
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Kidney transplantation in patients with kidney failure due to anti-GBM disease should be postponed until anti-GBM antibodies remain undetectable for ≥6 months
Media Gallery
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Goodpasture syndrome. A 45-year-old man was admitted to the intensive care unit with respiratory failure secondary to massive hemoptysis and acute renal failure. The antiglomerular basement membrane antibodies were strongly positive. The autopsy showed consolidated lung from extensive bleeding, which led to asphyxiation.
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Goodpasture syndrome. Close-up view of gross pathology in a 45-year-old man admitted to the intensive care unit with respiratory failure secondary to massive hemoptysis and acute renal failure. The antiglomerular basement membrane antibodies were strongly positive. The autopsy showed consolidated lung from extensive bleeding, which led to asphyxiation.
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Cytoplasmic antineutrophilic cytoplasmic antibodies (c-ANCA), which can appear in Goodpasture syndrome, are also commonly observed in Wegener granulomatosis and other vasculitides.
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Perinuclear antineutrophilic cytoplasmic antibodies (p-ANCA), which can appear in Goodpasture syndrome, are also observed in Churg-Strauss vasculitis and occasionally in Wegener granulomatosis.
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This is a renal biopsy slide of a patient who presented with hemoptysis and hematuria. The renal biopsy revealed crescentic glomerulonephritis, which may be caused by systemic lupus erythematosus, vasculitis, or Goodpasture syndrome.
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Goodpasture syndrome. A 35-year-old man who previously smoked cigarettes heavily, developed massive hemoptysis. The blood work showed positive anti–glomerular basement membrane antibodies.
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Immunofluorescence staining for immunoglobulin (IgG) reveals diffuse, high-intensity, linear staining of the glomerular basement membrane in a patient with anti–glomerular basement membrane (GBM) disease. Courtesy of Glen Markowitz, MD, Department of Pathology, Columbia University.
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