NDT Advance Access published online on April 26, 2005
Nephrology Dialysis Transplantation, doi:10.1093/ndt/gfh830
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1 Department of Nephrology, University Hospital, Bern, Switzerland; Department of Nephrology, Necker Hospital, Paris
* To whom correspondence should be addressed. Background. Pauci-immune renal vasculitis with focal glomerular necrosis and crescent formation is usually associated with anti-neutrophil cytoplasmic antibodies (ANCAs). However, ANCA's are absent in up to 10% of cases, which constitutes a rarely studied variant of renal vasculitis. Methods. This retrospective multicentre cohort study analyzed the presenting features, renal histology and outcome in 20 patients with pauci-immune crescentic necrotizing renal vasculitis in whom indirect immunofluorescence did not detect ANCA. Results. Renal histology revealed a high percentage of active glomerular lesions (50%), mainly cellular crescents, 28% of them with glomerular necrosis. Chronic tissue damage with glomerulosclerosis (21%) and diffuse interstitial fibrosis (40%) was already present at diagnosis, more prominent than in historical PR3-positive patients. Infiltrates of polymorphonuclear neutrophils in glomerular capillary loops were observed in 40% of all biopsies, mainly in necrotic lesions. The subsets of interstitially infiltrating leukocytes similar to ANCA-associated disease. Microscopic polyangiitis was diagnosed in 17 patients, Wegener's granulomatosis in two and renal-limited vasculitis in one. The patients median disease extent index (DEI) of 5 (range 4-11) reflected a systemic vasculitis. ANCA-negative vasculitis was not associated with infection or malignancy. Renal outcome was correlated to DEI (P = 0.032) and serum creatinine at diagnosis (P = 0.04). The mortality rate was high (35%) and closely related to age above 65 years at diagnosis (P = 0.014). Conclusions. The histological findings and prognosis in ANCA-negative renal vasculitis are comparable with those of ANCA-positive disease. Our data underline the importance of the exact diagnosis in an active vasculitic disease process even in the absence of ANCAs.
Received July 11, 2004
Accepted February 11, 2005
Original Articles
ANCA-negative pauci-immune renal vasculitis: histology and outcome
2 Department of Nephrology, Necker Hospital, Paris
3 Department of Internal Medicine, Valenciennes Hospital, France
4 INSERM U574, Necker Hospital, Paris
5 Department of Internal Medicine, Cochin Hospital, Paris
6 Department of Nephrology, University Hospital, Bern, Switzerland; INSERM U507, and AP-HP, Necker Hospital, Paris
7 INSERM U507, and AP-HP, Necker Hospital, Paris
Ute Eisenberger, E-mail: ute.eisenberger{at}insel.ch
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