Diagnostic Considerations
Conditions that affect the lung and kidney (pulmonary-renal syndromes) are important in the differential diagnosis. These include granulomatosis with polyangiitis (Wegener granulomatosis), systemic lupus erythematosus, microscopic polyangiitis, and other forms of systemic vasculitides. Distinguishing granulomatosis with polyangiitis from Goodpasture syndrome is particularly important. Interestingly, some patients with Goodpasture syndrome may present with antineutrophilic cytoplasmic antibodies (ANCAs), which are predominantly observed in patients with granulomatosis with polyangiitis. [23]
Pulmonary-renal syndromes are less commonly a manifestation of IgA-mediated disorders (eg, IgA nephropathy or Henoch-Schönlein purpura) and of immune complex–mediated renal disease (eg, essential mixed cryoglobulinemia). Rarely, rapidly progressive glomerulonephritis alone can cause pulmonary-renal syndromes through a mechanism involving renal failure, volume overload, and pulmonary edema with hemoptysis.
Careful attention to the medical history, physical examination, and targeted laboratory evaluation often suggests the underlying cause. Go to Pediatric Anti-GBM Disease (Goodpasture Syndrome) for complete information on this topic.
Differential Diagnoses
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Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome)
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Granulomatosis with Polyangiitis (GPA, formerly Wegener Granulomatosis)
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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.
