| ANCA-negative pauci-immune renal vasculitis: histology and outcome. | |
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MedLine Citation:
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PMID: 15855209 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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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. |
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Authors:
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Ute Eisenberger; Fadi Fakhouri; Philippe Vanhille; Hélène Beaufils; Alfred Mahr; Loic Guillevin; Philippe Lesavre; Laure-Hélène Noël |
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Publication Detail:
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Type: Journal Article; Multicenter Study Date: 2005-04-26 |
Journal Detail:
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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:
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Created Date: 2005-06-21 Completed Date: 2005-10-06 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 8706402 Medline TA: Nephrol Dial Transplant Country: England |
Other Details:
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Languages: eng Pagination: 1392-9 Citation Subset: IM |
Affiliation:
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University of Hospital, Department of Nephrology, Bern, Switzerland. ute.eisenberger@insel.ch |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Adult Aged Aged, 80 and over Antibodies, Antineutrophil Cytoplasmic / blood Cohort Studies Creatinine / blood Female Glomerulonephritis / blood, complications, pathology* Humans Male Middle Aged Outcome Assessment (Health Care) Prognosis Retrospective Studies Severity of Illness Index Vasculitis / blood, complications, pathology* |
| Chemical | |
Reg. No./Substance:
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0/Antibodies, Antineutrophil Cytoplasmic; 60-27-5/Creatinine |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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