Clinical History:

A 25-year-old man works in a military fuel depot. He began having respiratory difficulty along with red-tinged sputum. Two months later, he had increasing malaise and flank pain. He went to see the base physician. The patient then developed very rapid onset of renal failure with hematuria within three days.

Images 2.1 and 2.2:

What does the light microscopy show?

Crescents

Image 2.3:

What does the immunofluorescence show with staining for fibrinogen?

The crescent within Bowman's space stains for fibrinogen, consistent with injury leading to leakage of the fibrinogen that stimulates epithelial cell proliferation and crescent formation.

Image 2.4:

What does the immunofluorescence show with staining for IgG?

There is a linear pattern of staining along the glomerular basement membrane with IgG. In most cases of Goodpasture syndrome with rapidly progressive glomerulonephritis there is linear staining with IgG, but IgA and IgM can also be present.

Questions:

1. What tests and procedures would you order?
• Chest x-ray - infiltrates are present, but no cavitary lesions or mass lesions
• Sputum culture - negative for pathogens
• PPD - negative
• Urinalysis - many RBC's
2. What is the differential diagnosis?
• Goodpasture's syndrome
• Vasculitis (Wegener granulomatosis, SLE, polyarteritis nodosa)
• IgA nephropathy
3. What additional laboratory tests can be ordered?
• Anti-neutrophil cytoplasmic antibody (ANCA) - negative (tends to exclude Wegener's)
• anti-GBM antibody - positive

4. What are issues to consider if the patient, despite therapy, developed chronic renal failure? Should relatives be pressured to become allograft donors? What are issues involved with hemodialysis?

   Treatment options include chronic hemodialysis or a renal allograft. It is common to test many family members to find the best antigen match. But if one or more family members do not want to be a donor, what is said to them? Should the doctor lie to the patient (recipient) and say a particular relative was not a good match?

   Consider that dialysis requires going to a dialysis center 3 times a week, taking the better part of a morning or afternoon each time. Life has to be planned around these treatments. Diet and fluid intake must be carefully monitored. There are quality of life issues. Patients may feel worse before and after the time of the dialysis treatment and feel better between treatments. This continuous "up and down" cycle can be exhausting. Some dialysis patients adapt, but others find the treatment worse than the disease, and choose to stop dialysis. This will usually mean a coma in about one week and death in about two weeks, which is not a bad way to go. Stopping or minimizing fluid intake is essential of course, and pain medications should be made available as needed.