Dataset Information

Treatment and clinicopathological characteristics of lupus nephritis with anti-neutrophil cytoplasmic antibody positivity: a case-control study.

ABSTRACT:

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.

SUBMITTER: Li C

PROVIDER: S-EPMC5642651 | biostudies-literature | 2017 Jul

REPOSITORIES: biostudies-literature

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Publications

Treatment and clinicopathological characteristics of lupus nephritis with anti-neutrophil cytoplasmic antibody positivity: a case-control study.

Li Cui C Zhou Min-Lin ML Liang Dan-Dan DD Wang Jing-Jing JJ Yang Jing J Zeng Cai-Hong CH Liu Zhi-Hong ZH Zhang Hai-Tao HT

BMJ open 20170728 7

<h4>Objective</h4>To assess the clinical features, pathological presentations, treatments and outcomes of lupus nephritis (LN) with anti-neutrophil cytoplasmic antibody (ANCA) positivity.<h4>Design</h4>A case-control study.<h4>Methods</h4>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=12 ...[more]

PMID: 28756384

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Project description:BACKGROUND:Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) comprises microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA) and eosinophilic GPA (EGPA). Myeloperoxidase (MPO) and proteinase 3 (PR3) are the main antigens for ANCA. AAV is a common multisystem autoimmune disease and most of the studies on AAV have been conducted in Western countries. Nowadays in China many efforts are made to investigate this disease. SUMMARY:This review highlights the progress in the prevalence, management and outcomes of AAV in Chinese patients. With respect to the prevalence of AAV, though there are no precise data, AAV is not rare in the Chinese population. In Chinese patients with AAV there is a striking preponderance of MPA, and MPO-ANCA is much more common than PR3-ANCA. Even in patients with GPA there is a predominance of MPO-ANCA over PR3-ANCA. Propylthiouracil-induced AAV and ANCA-negative pauci-immune glomerulonephritis are stated in this review as well. With respect to the management of AAV, glucocorticoids in combination with cyclophosphamide remain the mainstay of induction therapy. Besides, we describe predictors of different outcomes in Chinese patients, including mortality, relapse, treatment resistance and end-stage renal disease. KEY MESSAGES:AAV is not rare in the Chinese population. The disease spectrum and subtypes of ANCA are different between patients with AAV in China and Western countries. The treatment strategy for AAV in China is in consistency with that in Western countries. Predictors of different clinical outcomes are provided. FACTS FROM EAST AND WEST:Treatment options for AAV are shared between the East and West, with corticosteroid combined with cyclophosphamide being the standard regimen for inductive therapy and switching to azathioprine after remission. The major cause of death in treated patients is infection related to immunosuppressive therapy within the first year after diagnosis, and this rate might be higher in China than in Western countries. Western studies demonstrated the efficacy and safety of rituximab for induction of remission in cases with relatively mild disease and maintenance therapy, but this agent is rarely used in China.

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Dataset Information

Treatment and clinicopathological characteristics of lupus nephritis with anti-neutrophil cytoplasmic antibody positivity: a case-control study.

Objective

Design

Methods

Results

Conclusions

Publications

Treatment and clinicopathological characteristics of lupus nephritis with anti-neutrophil cytoplasmic antibody positivity: a case-control study.

Similar Datasets

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