Clinical features of Goodpasture's disease
Rapidly progressive renal failure with an active urinary sediment containing protein, red blood cells, and red cell casts. Systemic
In 1919, Goodpasture was attempting to define the specific pathological features of influenza infection in diseased lungs, and reported the autopsy findings in two patients who had died at the height of the 1918–19 pandemic. The finding of hyaline membranes overlying dilated alveoli, without positive microbiological cultures, was thought to be pathognomonic of influenza. However, one of the patients had evidence of systemic disease with prominent renal involvement.1 This description of a
Antibodies to GBM are seen in the kidney and can generally be detected in the circulation. They are mostly IgG, although IgA and IgM antibodies have been reported. In most cases they are directed against the non-collagenous region of the α3 chain of type IV collagen, a molecule with restricted tissue distribution and found only in specialised basement membranes, including those of the renal glomerulus and the alveoli.
Goodpasture's disease predominantly affects Caucasian populations, with
Before supportive dialysis therapy and plasmapheresis, the outcome of this condition was universally poor. Recognition that presence of antibodies to GBM might be pathogenic led to therapeutic strategies to remove circulating antibody. Current expert management can ensure that over 90% of patients survive the acute illness.
Clinical features of Goodpasture's disease Rapidly progressive renal failure with an active urinary sediment containing protein, red blood cells, and red cell casts. Systemic
The idea that antibodies to GBM might be pathogenic dates back to 1967 when Lerner, Glassock, and Dixon, in a classic series of experiments, transferred antibodies eluted from kidneys of patients with Goodpasture's disease to squirrel monkeys, which subsequently developed glomerulonephritis.9 Rapid disease recurrence occurs if transplantation is done in the presence of circulating antibody to GBM and correlations have been reported between disease severity and antibody litre.8 As a result, much
Ernest Goodpasture (1886–1960) was a highly acclaimed pathologist, scientist, and teacher, whose pioneering work in virology paved the way for production of vaccines and mass immunisation programmes during the Second World War (figure 4).12 His interest in pathology began during his training at Johns Hopkins Medical School, Baltimore, MD, USA, studying under George H Whipple. His first publication, in 1913, was written in collaboration with Whipple, on the subject of haemorrhagic pancreatitis.