Objective To assess the clinical features, pathological presentations, treatments and outcomes of lupus nephritis (LN) with anti-neutrophil cytoplasmic antibody (ANCA) positivity.
Design A case–control study.
Methods Patients (n=49) were retrospectively included from Jinling Hospital in China if presenting with biopsy-proven ANCA-positive LN between 1985 and 2008. Clinicopathological characteristics and outcomes were analysed and compared with those of a control group (n=1279). We further compared treatment responses and outcomes of ANCA-positive LN patients based on the treatment issued.
Results The study included 40 women and 9 men (median age 33 years at biopsy): 38 with myeloperoxidase (MPO)-ANCA, 7 with proteinase 3 (PR3)-ANCA and 4 with double positivity. ANCA-positive LN patients exhibited higher haematuria, serum creatinine levels and systemic lupus erythematosus disease activity index scores. On pathological evaluation, class IV LN was predominant, accounting for 61.22% of cases. Light microscopy revealed significantly higher activity index and chronicity index scores, including cellular crescents, interstitial inflammation, tubular atrophy and interstitial fibrosis. ANCA-positive LN patients receiving mycophenolate mofetil as induction therapy had a higher remission rate and better renal outcomes than those receiving cyclophosphamide. During follow-up, end-stage renal disease developed in seven (14.29%) ANCA-positive LN patients, all of them were MPO-ANCA positive.
Conclusions The characteristics of ANCA-positive LN were massive haematuria and advanced renal insufficiency. We observed a higher remission rate and better prognoses when using mycophenolate mofetil than when using cyclophosphamide as induction therapy.
- Antineutrophil Cytoplasmic Autoantibody
- Clinical Features
- Lupus Nephritis
- Pathological Presentations
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Contributors All authors contributed significantly to this manuscript, including contributions in the study design (CL and HTZ), chart reviews (CL, MLZ, DDL, ZHL and HTZ), interpretation of the data (CL, MLZ, DDL and HTZ) and preparation of the manuscript (CL, MLZ, DDL, JJW, JY, CHZ, ZHL and HTZ).
Funding This study was supported by the National Key Technology Research and Development Program (No. 2013BAI09B04 and No. 2015BAI12B05) and the Jiangsu Provincial Clinical Science and Technology Project (No BL2012007).
Competing interests None declared.
Ethics approval Jinling Hospital, Nanjing University School of Medicine, China.
Provenance and peer review Not commissioned; externally peer reviewed.
Data sharing statement We will not share data.
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