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76 A RARE CASE OF RAPIDLY PROGRESSIVE GLOMERULONEPHRITIS POSITIVE FOR BOTH ANTIGLOMERULAR BASEMENT MEMBRANE ANTIBODY AND PERINUCLEAR ANTINEUTROPHILIC CYTOPLASMIC ANTIBODY.
  1. A. J. Simpson
  1. Emory University, Atlanta, GA

Abstract

The case presented is of a 50-year-old female with a chief complaint of weakness, nausea, and vomiting. She was found to be in acute renal failure with a creatinine of 10.0, and urinalysis showed dysmorphic red blood cells. Renal biopsy showed extensive glomerular crescents suggestive of rapidly progressive glomerulonephritis. Follow-up tests revealed that she was positive for both anti-GBM (glomerular basement membrane) antibodies and p-ANCA (antineutrophilic cytoplasmic antibody). She presented late in the disease process with very poor renal function, and despite aggressive treatment, she remained dialysis dependent 4 months after diagnosis. This patient represents a rare presentation of rapidly progressive glomerulonephritis with concomitant presence of both anti-GBM antibodies and p-ANCA. Anti-GBM or Goodpasture's sydrome is a rare disease, with only approximately 30% of these patients also being positive for ANCA, most commonly p-ANCA. Alternatively, about 5% of patients positive for p-ANCA are anti-GBM positive. The significance of recognizing these patients with this presentation lies in the clinical outcome. Patients with pure anti-GBM rarely recover from severe renal disease, whereas up to 75% of those with ANCA-associated vasculitis can recover renal function even when presenting with acute renal failure that requires dialysis. Patients who are positive for both antibodies have survival and renal recovery rates much more similar to anti-GBM disease than ANCA-associated vasculitis. As a result, these patients benefit from more aggressive treatment than is standard for ANCA-associated vasculitis alone. Therefore, it may be prudent to test patients diagnosed with either disease for the other antibody to guide therapy and outcome.

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