History
Anti–glomerular basement membrane (anti-GBM) antibody disease can occur year-round, but the incidence increases in the spring and in early summer. Most patients present with features of systemic illness and either acute nephritis or pulmonary involvement; a subset may have all 3 findings. Pulmonary involvement can precede the onset of glomerulonephritis by several years, or it can develop after renal disease is evident.
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Symptoms of systemic illness include low-grade fever, malaise, headache, anorexia, nausea, vomiting, weight loss, and fatigue.
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Symptoms of renal involvement include hematuria, oliguria, and edema.
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Symptoms of pulmonary involvement include shortness of breath, cough, and expectoration of material that ranges from blood-streaked sputum to massive hemoptysis. A feeling of warmth inside the chest may precede hemoptysis.
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Approximately 30% of patients with Goodpasture syndrome are antineutrophilic cytoplasmic antibody (ANCA) positive some time during the illness. Such patients may present with pruritic skin rashes and arthralgia.
Physical Examination
Physical findings depend on the organ system involved and on the severity of the disease. No abnormalities may be evident in the absence of renal or pulmonary involvement.
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Signs of renal involvement include the following:
When renal involvement is severe, volume overload of the intracellular and extracellular fluid may result in tachycardia, tachypnea, hypertension, pulmonary rales, and pitting edema.
Patients with uremia may have a specific breath odor, bruises, pallor, tremor, myoclonus, asterixis, focal neurologic signs, mental status changes, and seizures.
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Signs of pulmonary involvement include the following:
Respiratory distress ranging from mild distress to respiratory failure
Pulmonary hemorrhage that may result in pallor, tachycardia, and shock
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Pulmonary manifestations can precede or follow signs of nephritis.
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Patients with high ANCA and low anti-GBM antibody titers may present with various vasculitic skin rashes.
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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.
