History
Substantial variation exists in the clinical manifestations of patients with anti–glomerular basement membrane (anti-GBM) disease. From 60-80% of patients have clinically apparent manifestations of pulmonary and renal disease, 20-40% have renal disease alone, and less than 10% have disease that is limited to the lungs.
Symptoms include the following:
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Constitutional symptoms (eg, malaise, chills and fever, arthralgias) may precede or develop concurrently with pulmonary or renal manifestations
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Hemoptysis is the presenting sign when the disease affects the lungs. The level of hemoptysis may vary and, in a small percentage of patients, may be absent. Other pulmonary manifestations include cough, dyspnea, and shortness of breath
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Massive pulmonary hemorrhage leading to respiratory failure may occur
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Chest pain is present in less than half of the patients
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Renal manifestations include hematuria, edema, high blood pressure, and eventually uremia
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Significant anemia may result from persistent intrapulmonary bleeding
Physical Examination
Physical examination findings in patients with anti-GBM disease include the following:
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Tachypnea
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Inspiratory crackles over lung bases
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Cyanosis
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Hepatosplenomegaly (may be present)
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Hypertension (present in 20% of cases)
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Rash
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Edema
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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.
