NDT Advance Access originally published online on April 26, 2005
Nephrology Dialysis Transplantation 2005 20(7):1392-1399; doi:10.1093/ndt/gfh830
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ANCA-negative pauci-immune renal vasculitis: histology and outcome
1 Department of Nephrology, University Hospital, Bern, Switzerland, 2 Department of Nephrology, 4 INSERM U574, 6 INSERM U507, and AP-HP, Necker Hospital, 5 Department of Internal Medicine, Cochin Hospital, Paris and 3 Department of Internal Medicine, Valenciennes Hospital, France
Correspondence and offprint requests to: Ute Eisenberger, MD, University of Bern Inselspital, Department of Nephrology, Freiburgstr. 4, 3010 Bern, Switzerland. Email: ute.eisenberger{at}insel.ch
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.
Keywords: ANCA-negative; microscopic polyangiitis; neutrophils; pauci-immune renal vasculitis; renal biopsy; renal outcome; Wegener's granulomatosis
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