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Nephrol Dial Transplant (2002) 17: 587-596
© 2002 European Renal Association-European Dialysis and Transplant Association

Long-term renal injury in ANCA-associated vasculitis: an analysis of 31 patients with follow-up biopsies

Herbert A. Hauer1,, Ingeborg M. Bajema4, E. Christiaan Hagen5, Laure-Hélène Noël6, Franco Ferrario7, Rüdiger Waldherr8, Hans C. van Houwelingen2, Philippe Lesavre6, Renato A. Sinico7, Fokko van der Woude9, Gill Gaskin10, Cornelis A. Verburgh3, Emile de Heer1 and Jan A. Bruijn1

1 Department of Pathology, 2 Department of Medical Statistics and 3 Department of Nephrology, Leiden University Medical Center, Leiden, The Netherlands, 4 Department of Pathology, Erasmus University Medical Center Rotterdam, The Netherlands, 5 Department of Internal Medicine, Eemland Hospital, Amersfoort, The Netherlands, 6 Department of Nephrology and INSERM U507, Hôpital Necker, Paris, France, 7 Renal Immunopathology Center, Ospedale San Carlo Borromeo, Milan, Italy, 8 Department of Pathology, University of Heidelberg, Heidelberg, Germany, 9 Department of Nephrology, University of Heidelberg, Mannheim, Germany and 10 Renal Section, Hammersmith Hospital, London, UK

Background. We reported previously that in renal disease in relation to antineutrophil cytoplasm auto-antibodies (ANCA)-associated vasculitis, renal outcome correlates better with the percentage of normal glomeruli than with separate active lesions. This may imply that glomeruli, once affected by necrotizing and crescentic lesions, are irreversibly damaged. We quantified and evaluated the course of renal lesions in the present study.

Methods. We retrospectively analysed 31 patients with renal disease in relation to ANCA-associated vasculitis, all treated with immunosuppressive drugs. In all patients, a renal biopsy was performed at diagnosis. A follow-up biopsy was performed in all patients on the indication of a suspected renal relapse, after a mean interval of 31 months.

Results. The mean percentage of normal glomeruli in the renal biopsy did not change over time (29% in the initial and 30% in the follow-up biopsy). The mean percentage of glomeruli with crescents, however, significantly decreased from 57 to 30% (P<0.001). The percentage of glomerulosclerosis significantly increased from 12 to 39% (P<0.001). The data were independent of diagnosis, gender, age, time interval between the biopsies, and treatment.

Conclusions. This is the first study to quantify glomerular changes between two time points in patients with renal vasculitis. Our results suggest that, on average, no new glomeruli are recruited into the active disease process. The sum of the percentage of crescentic and sclerotic glomeruli in the initial biopsies is larger than the percentage of sclerotic glomeruli in the follow-up biopsies. Thus, therapy seems not only to prevent normal glomeruli from being recruited into the active disease process for a certain time, but seems also to allow part of the active lesions to revert into a normal phenotype, although another part of the active lesions will be transformed to a chronic phenotype.

Keywords: ANCA-associated vasculitis; follow-up biopsies; microscopic polyangiitis; pauci-immune crescentic necrotizing glomerulonephritis; renal limited vasculitis; Wegener's granulomatosis

Correspondence and offprint requests to: Herbert A. Hauer, Leiden University Medical Center, Department of Pathology, Building 1, L1-Q, PO Box 9600, NL-2300 RC Leiden, the Netherlands. Email: H.A.Hauer{at}LUMC.nl


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