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Clinical and Pathological Study on Patients With Primary Antineutrophil Cytoplasmic Autoantibody–Associated Vasculitis With Renal Immune Complex Deposition

Li, Xin MD; Zhang, Wen MD, PhD; Yu, Hai-Jing MD, PhD; Pan, Xiao-Xia MD, PhD; Shen, Ping-Yan MD; Ren, Hong MD; Wang, Wei-Ming MD, PhD; Chen, Nan MD, PhD

JCR: Journal of Clinical Rheumatology: January 2015 - Volume 21 - Issue 1 - p 3–9
doi: 10.1097/RHU.0000000000000207
Original Articles
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Background Traditionally, antineutrophil cytoplasmic autoantibody–associated vasculitis (AAV) is histologically characterized by pauci-immune glomerulonephritis. However, more and more literature has reported immune complex (IC) deposits to be found in renal specimen from patients with AAV. The role that these IC deposits play in the development of AAV, as well as their clinical and pathological significance, is worthy of studying.

Objectives The objective of this study was to analyze the clinical and pathological characteristics of Chinese patients with AAV having renal IC deposition.

Methods A retrospective study was performed on 34 patients with AAV in Shanghai Ruijin Hospital with renal IC deposition. Clinical and pathological data were collected and studied and compared with other 76 AAV patients having classic pauci-immune glomerulonephritis.

Results Thirty-four patients were enrolled in this study, with a mean age of 56.4 ± 16.4 years and a male-female ratio of 1:1.3 (19/15). Twenty-seven patients (79.4%) had impaired renal function, with an average serum creatinine of 4.4 ± 3.2 mg/dL. C3 (82.4%) and immunoglobulin M (50%) were the most common IC deposits observed in the kidneys. During the follow-up (median, 39 months), 6 patients (17.7%) died, and 11 (32.4%) finally progressed to end-stage renal disease despite immunosuppressive therapy. Compared with patients having classic pauci-immune glomerulonephritis, patients with renal IC deposits had similar clinical and laboratory features except for more proteinuria (2374 ± 2221 vs 1444 ± 1956 mg/24 h, P = 0.002), a higher prevalence of nephrotic syndrome (30.3% vs 9.6%, P = 0.007) and hypocomplementemia (86.8 ± 33.1 vs 110 ± 45.5 mg/dL, P = 0.029), and also a higher risk for progressing to end-stage renal disease (32.4% vs 13.1%, P = 0.018).

Conclusions Patients with AAV with renal IC deposition might have a worse renal prognosis than those having classic pauci-immune glomerulonephritis.

From the Department of Nephrology, Shanghai Jiaotong University Affiliated Ruijin Hospital, Shanghai, China.

Supported by the National key technology R&D Program (12-5), 2011BAI10B00 (2011BAI10B06), for research on hypertensive nephropathy and ischemic kidney diseases; and Shanghai Municipal Science and Technology Commission, 10411965900, to improve early diagnosis of acute kidney injury.

The authors declare no conflict of interest.

Correspondence: Nan Chen, MD, PhD, Department of Nephrology, Shanghai Ruijin Hospital, School of Medicine, Shanghai Jiaotong University, Shanghai 200025, People’s Republic of China. E-mail: chen-nan@medmail.com.cn.

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