Journal of the American Society of Nephrology
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Published ahead of print on January 10, 2007
J Am Soc Nephrol 18: 599-605, 2007
© 2007 American Society of Nephrology
doi: 10.1681/ASN.2006091021

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Clinical Nephrology

Antineutrophil Cytoplasmic Autoantibody–Negative Pauci-immune Crescentic Glomerulonephritis

Min Chen, Feng Yu, Su-Xia Wang, Wan-Zhong Zou, Ming-Hui Zhao and Hai-Yan Wang

Renal Division and Institute of Nephrology, Peking University First Hospital, Beijing, People’s Republic of China

Address correspondence to: Dr. Ming-Hui Zhao, Renal Division and Institute of Nephrology, Peking University First Hospital, Beijing 100034, P.R. China. Phone: +86-10-66551736; Fax: +86-10-66551055; E-mail: mhzhao{at}bjmu.edu.cn

Received for publication September 19, 2006. Accepted for publication November 24, 2006.

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.


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