Document Detail

ANCA-negative pauci-immune renal vasculitis: histology and outcome.
MedLine Citation:
PMID:  15855209     Owner:  NLM     Status:  MEDLINE    
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.
Ute Eisenberger; Fadi Fakhouri; Philippe Vanhille; Hélène Beaufils; Alfred Mahr; Loic Guillevin; Philippe Lesavre; Laure-Hélène Noël
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Publication Detail:
Type:  Journal Article; Multicenter Study     Date:  2005-04-26
Journal Detail:
Title:  Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association     Volume:  20     ISSN:  0931-0509     ISO Abbreviation:  Nephrol. Dial. Transplant.     Publication Date:  2005 Jul 
Date Detail:
Created Date:  2005-06-21     Completed Date:  2005-10-06     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8706402     Medline TA:  Nephrol Dial Transplant     Country:  England    
Other Details:
Languages:  eng     Pagination:  1392-9     Citation Subset:  IM    
University of Hospital, Department of Nephrology, Bern, Switzerland.
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MeSH Terms
Aged, 80 and over
Antibodies, Antineutrophil Cytoplasmic / blood
Cohort Studies
Creatinine / blood
Glomerulonephritis / blood,  complications,  pathology*
Middle Aged
Outcome Assessment (Health Care)
Retrospective Studies
Severity of Illness Index
Vasculitis / blood,  complications,  pathology*
Reg. No./Substance:
0/Antibodies, Antineutrophil Cytoplasmic; 60-27-5/Creatinine

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