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

BMJ Open. 2017 Jul 28;7(7):e015668. doi: 10.1136/bmjopen-2016-015668.

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.

Keywords: Antineutrophil Cytoplasmic Autoantibody; Clinical Features; Lupus Nephritis; Outcome; Pathological Presentations; Treatment.

Publication types

  • Comparative Study

MeSH terms

  • Adult
  • Antibodies, Antineutrophil Cytoplasmic
  • Case-Control Studies
  • China / epidemiology
  • Cyclophosphamide / therapeutic use*
  • Female
  • Hematuria / drug therapy*
  • Hematuria / epidemiology
  • Hematuria / physiopathology
  • Humans
  • Immunosuppressive Agents / therapeutic use*
  • Lupus Nephritis / drug therapy*
  • Lupus Nephritis / epidemiology
  • Lupus Nephritis / physiopathology
  • Male
  • Mycophenolic Acid / therapeutic use*
  • Remission Induction
  • Renal Insufficiency / drug therapy*
  • Renal Insufficiency / epidemiology
  • Renal Insufficiency / physiopathology
  • Retrospective Studies
  • Treatment Outcome

Substances

  • Antibodies, Antineutrophil Cytoplasmic
  • Immunosuppressive Agents
  • Cyclophosphamide
  • Mycophenolic Acid