Abstract
Goodpasture's disease is a fulminant rapidly progressive disease characterized by autoantibodies to the alpha-3 chain of type IV collagen (Goodpasture's antigen). It can present as a glomerulonephritis alone, or as a pulmonaryrenal syndrome with alveolar haemorrhage. We report here a classical case of Goodpasture's disease presenting as a pulmonary-renal syndrome with serum anti-glomerular basement membrane (GBM) antibody negativity and positive perinuclear anti-neutrophil cytoplasm antibodies (p-ANCA).
Highlights
Goodpasture’s disease is a fulminant rapidly progressive disease characterized by autoantibodies to the alpha-3 chain of type IV collagen (Goodpasture’s antigen) [1]
It can present as a glomerulonephritis alone, or as a pulmonary-renal syndrome with alveolar haemorrhage [2]
Serum creatinine stabilization and the opinion of an expert nephrologist were relied upon to determine the duration of treatment
Summary
Goodpasture’s disease is a fulminant rapidly progressive disease characterized by autoantibodies to the alpha-3 chain of type IV collagen (Goodpasture’s antigen) [1]. We report here a classical case of Goodpasture’s disease presenting as a pulmonary-renal syndrome with serum anti-glomerular basement membrane (GBM) antibody negativity and positive perinuclear anti-neutrophil cytoplasm antibodies (p-ANCA). The patient was a lifelong non-smoker, and there were no relevant occupational or domestic exposures He had no significant medical history except for a similar episode of shortness of breath a year previously that had been diagnosed by his general practitioner as post-infectious bronchospasm. Serial anti-GBM antibody titers could not be measured in this case it was difficult to determine the duration and role of plasmapharesis in this patient His clinical improvement, serum creatinine stabilization and the opinion of an expert nephrologist were relied upon to determine the duration of treatment.
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