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| Main Authors: | , , , , |
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| Format: | Recurso digital |
| Language: | |
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Zenodo
2026
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| Subjects: | |
| Online Access: | https://doi.org/10.5281/zenodo.18375854 |
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Table of Contents:
- <p><span>Pulmonary-renal syndrome associated with Goodpasture disease is a rare but life-threatening condition characterized by the concurrent presence of diffuse alveolar hemorrhage and rapidly progressive glomerulonephritis. Initially described in 1919 as an apparent post-infectious process, the disease is now recognized as a distinct autoimmune disorder caused by circulating anti-glomerular basement membrane antibodies directed against the α3 chain of type IV collagen. This antigen is shared by the glomerular and alveolar basement membranes, explaining the characteristic dual organ involvement. Advances in immunopathology have established anti-GBM disease as a form of small-vessel vasculitis, with linear immunoglobulin G deposition along the glomerular basement membrane representing a pathognomonic histological feature. The pathogenesis involves loss of immune tolerance in genetically susceptible individuals, particularly those carrying specific human leukocyte antigen alleles, with environmental factors such as smoking and infections acting as potential triggers. Renal involvement typically manifests as crescentic rapidly progressive glomerulonephritis, which is strongly associated with poor renal outcomes and progression to end-stage kidney disease. Pulmonary involvement, present in approximately half of patients, is dominated by diffuse alveolar hemorrhage and may lead to acute respiratory failure. Diagnosis relies on serological detection of anti-GBM antibodies, assessment of renal function and urinalysis, imaging for pulmonary hemorrhage, and confirmation by kidney biopsy. Management requires urgent stabilization and early initiation of combined immunosuppressive therapy with glucocorticoids and cyclophosphamide, often complemented by therapeutic plasma exchange to remove circulating pathogenic antibodies. Prognosis is closely linked to disease severity at presentation, particularly renal function, respiratory failure, and antibody status. Although outcomes have improved with modern therapy, Goodpasture disease remains associated with significant morbidity and mortality, underscoring the importance of early recognition, accurate diagnosis, and prompt, aggressive treatment.</span></p>