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Risk of malignancy in patients treated for systemic necrotising vasculitis
  1. Lafarge Antoine1,2,
  2. Adrien Joseph3,
  3. Christian Pagnoux4,
  4. Xavier Puechal1,2,
  5. Pacal Cohen1,2,
  6. Maxime Samson5,
  7. Mohamed Hamidou6,
  8. Alexandre Karras7,
  9. Matthieu Groh1,
  10. Thomas Quemeneur8,
  11. Camillo Ribi9,
  12. Luc Mouthon1,2,10,
  13. Loic Guillevin1,2,10,
  14. Benjamin Terrier1,2,10
  15. on behalf of the French Vasculitis Study Group (FVSG)
  1. 1Department of Internal Medicine, Cochin Hospital, Paris, France
  2. 2National Referral Center for Rare Systemic Autoimmune Diseases, Cochin Hospital, Paris, France
  3. 3U1138, INSERM, Paris, France
  4. 4Department of Rheumatology, Mount Sinai Hospital, Toronto, Ontario, Canada
  5. 5Department of Internal Medicine and Clinical Immunology, Hôpital François Mitterrand, Dijon, France
  6. 6Department of Internal Medicine, Hôtel Dieu, Nantes, France
  7. 7Department of Nephrology, Hôpital Européen Georges Pompidou, Paris, France
  8. 8Department of Internal Medicine, Hôpital de Valenciennes, Valenciennes, France
  9. 9Department of Immunology, CHUV, Lausanne, Switzerland
  10. 10Paris Descartes University, Paris, France
  1. Correspondence to Dr Lafarge Antoine, Internal Medicine, Hopital Cochin, Paris 75014, France; antoinelafarge{at}; Pr Benjamin Terrier; benjamin.terrier{at}

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Reduction in cyclophosphamide cumulative dose and introduction of newer immunosuppressive drugs may reduce the malignant burden of systemic necrotising vasculitis (SNV).1 2 This study aimed to describe malignancies recorded in five randomised controlled trials in SNV conducted by the French Vasculitis Study Group and to identify predictive factors.

CHUSPAN, CHUSPAN 2, WEGENT, CORTAGE and MAINRITSAN trials evaluated different therapeutic strategies, summarised in online supplementary table S1, for the treatment of newly diagnosed or relapsing SNV. Informations regarding methods and references are provided in the online supplementary materials. The primary endpoint was the occurrence of malignancy.

Supplemental material


A total of 733 patients included between 1993 and 2012 were pooled. Baseline characteristics of the population are summarised in table 1. During a 4485.9 person-years (PY) observation period, 39 (5.3%) patients developed malignancies (869.5 per 100 000 PY), including solid cancers in 34 (4.6%) cases (757.9 per 100000 PY) and …

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  • Handling editor Josef S Smolen

  • Contributors LA and BT devised the project, the main conceptual ideas and proof outline, pooled and analysed data from the five randomised controlled trials (RCTs). AJ performed the statistical analysis. CP, XP, PC, CR, LG and BT designed and published the five RCTs involved in this article. LA and BT, wrote the manuscript. All authors discussed the results and contributed to the final manuscript.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Patient consent for publication Not required.

  • Provenance and peer review Not commissioned; externally peer reviewed.