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Case Studies: The Kidney, Urinary Bladder and Testis: Case 2: Goodpasture Syndrome
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A 35-year old man presented with massive hemoptysis and blood in urine. Over the next two days he developed complete anuria and was placed on an artificial kidney machine. He then underwent plasmapheresis and improved considerably. However, even though his pulmonary symptoms disappeared, his renal functions never returned to normal, and he underwent renal transplantation. He was discharged two months later in stable condition.
Light microscopic findings. Why is this condition called crescentic glomerulonephritis?
A glomerulus with a crescent is illustrated. What are the cells forming the crescent? What happened with the capillary loops? Why do patients become anuric?
Immunofluorescence microscopy shows linear deposits of IgG along the glomerular basement membrane.
In order to determine whether the patient's serum contains anti-glomerular basement membrane antibodies, his serum was drawn and layered over the section of a normal test kidney. After the incubation with patient's serum, the non-bound serum was washed away and the sections were covered with a fluorescein tagged rabbit anti-human IgG antiserum. This slide shows that the glomerular basement membrane fluorescence, in a pattern similar to the patient's own glomeruli. Is this proof that the patient has circulating auto-antibodies to glomerular basement membrane? Would a similar test be positive with sections of normal lung?
The diagnosis is Goodpasture syndrome.
Lung biopsy shows massive intraalveolar hemorrhage due to the effects of anti-basement membrane antibody.
Question for Class Discussion
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