Case report
Sequential development of perinuclear ANCA-associated vasculitis and anti-glomerular basement membrane glomerulonephritis

https://doi.org/10.1053/j.ajkd.2003.11.019Get rights and content

Abstract

A 75-year-old man suffered from perinuclear antineutrophil cytoplasm antibody (p-ANCA)-associated vasculitis with mild renal involvement. Three years later, he suddenly experienced an anuric acute renal failure due to anti-glomerular basement membrane (GBM) disease. Antibodies to myeloperoxydase were continuously present at a high titer in the patient's serum while serum anti-GBM antibodies were only detected at the time of the acute renal failure. A substantial proportion of patients with anti-GBM glomerulonephritis simultaneously display ANCAs whose pathogenic role is not clear. In our case, ANCAs were supposed to be of pathogenic importance because they may have uncovered the Goodpasture antigen. This case report lends further support to the concept that p-ANCA vasculitis may trigger anti-GBM disease.

Section snippets

Case report

A 75-year-old man was admitted to the department of nephrology in January 2003 for anuric acute renal failure. He was treated in the internal medicine department from March 2000, and a p-ANCA-associated vasculitis was diagnosed. He had no previous medical history. When first seen he was suffering from intense myalgias of the lower limbs and sensitivity of the calves, with weight loss and moderate fever. Clinical examination revealed mild livedo reticularis. Blood pressure was 140/90 mm Hg.

Discussion

We report the case of a 72-year-old man who sequentially developed a p-ANCA-associated vasculitis, followed 3 years later by Goodpasture syndrome, revealed by an acute renal failure. Plasma exchanges, high-dose steroids, and cyclophosphamide failed to improve renal function but induced the disappearance of p-ANCAs and anti-GBM antibodies.

During the first part of his medical history, our patient presented a p-ANCA-associated vasculitis that fulfilled the classification criteria for microscopic

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