Periglomerular granulomatosis. A limited form of Wegener's granulomatosis with exclusive renal involvement?

Three episodes of acute renal failure, all of them requiring hemodialysis, were observed in two patients. The renal biopsy specimens showed a massive periglomerular granulomatosis with crescentic glomerulonephritis. With cyclophosphamide therapy, a dramatic improvement in renal function was observed in both patients. Although the histologic findings and the response to cyclophosphamide therapy were characteristic of Wegener's granulomatosis, there was no evidence of respiratory involvement or extrarenal manifestations throughout the patients' clinical course. We think that these cases could represent limited forms of Wegener's granulomatosis with exclusive renal involvement.

[Rapidly progressive glomerular disease treated with plasmapheresis (author's transl)]. / Glomerulonefritis rápidamente progresiva tratada con plasmaféresis.

A case of rapidly progressive glomerular disease with 100% of epithelial crescents completely enveloping each glomerulus, prolonged anuria and renal failure requiring dialysis. There were no indications of either streptococcal etiology or systemic disease. Improvement in renal function in response to plasmapheresis and immunosuppressants was spectacular. During two subsequent episodes of renal failure with diminished diuresis response to plasmapheresis was again striking. Assay for circulating immune-complex was always negative. No anti-basement membrane antibodies were found. Due to the lack of an adequate vascular access plasmapheresis was discontinued and the patient died 7 months after onset. Tendency to interstitial glomerular sclerosis was established with three renal biopsy specimens taken during progression of the disease. Intense metabolic acidosis suggestive of tubular acidosis, disproportionate to renal insufficiency presented during the last months. Pathologic and prognostic aspects as well as possible access for plasmapheresis are discussed.