title: Antineutrophil cytoplasmic autoantibody-negative Pauci-immune crescentic glomerulonephritis.
abstract: Pauci-immune crescentic glomerulonephritis (CrGN) is one of the most common causes of rapidly progressive glomerulonephritis. The majority of patients with pauci-immune CrGN had circulating antineutrophil cytoplasmic autoantibody (ANCA). However, patients with ANCA-negative pauci-immune CrGN were not investigated fully. This study aimed to analyze the characteristics of this subgroup of patients. Patients whose pauci-immune CrGN was diagnosed from 1997 to 2006 in one center were studied retrospectively. The criteria of pauci-immune was defined as "the intensity of glomerular immunoglobulins staining by direct immunofluorescence assay in renal sections was negative to 1+ staining on a scale of 0 to 4+." Clinical and pathologic characteristics were compared between patients with and without ANCA. Among the 85 patients with pauci-immune CrGN, 28 (32.9%) were ANCA negative. Compared with the 57 ANCA-positive patients, the ANCA-negative patients were much younger (39.7 +/- 17.0 versus 57.6 +/- 14.0 yr; P < 0.001). The level of urinary protein and the prevalence of nephrotic syndrome were significantly higher in ANCA-negative patients than that in ANCA-positive patients (P < 0.01 and P < 0.001, respectively). However, the prevalence of extrarenal involvement was significantly lower in ANCA-negative patients than that in ANCA-positive patients. The renal survival was poorer in ANCA-negative patients than that in ANCA-positive ones (P < 0.05). ANCA-negative pauci-immune CrGN was not rare and might represent an independent disease entity from ANCA-positive vasculitis.
authors.affiliation: Renal Division and Institute of Nephrology, Peking University First Hospital, Beijing 100034, P.R. China. mhzhao@bjmu.edu.cn
authors.forename: Min
authors.initials: M
authors.lastname: Chen
authors.forename: Feng
authors.initials: F
authors.lastname: Yu
authors.forename: Su-Xia
authors.initials: SX
authors.lastname: Wang
authors.forename: Wan-Zhong
authors.initials: WZ
authors.lastname: Zou
authors.forename: Ming-Hui
authors.initials: MH
authors.lastname: Zhao
authors.forename: Hai-Yan
authors.initials: HY
authors.lastname: Wang
mesh_terms: D000328:Adult; D017668:Age of Onset; D000368:Aged; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000276:immunology*; D000918:Antibody Specificity; D004797:Enzyme-Linked Immunosorbent Assay; D005260:Female; D005921:Glomerulonephritis / Q000276:immunology* / Q000473:pathology* / Q000652:urine; D006801:Humans; D007668:Kidney / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D011507:Proteinuria
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic