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Microscopic polyangiitis and non-HBV polyarteritis nodosa with poor-prognosis factors: 10-year results of the prospective CHUSPAN trial


1, 2, 3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14, 15

 

  1. Dept. of Internal Medicine, Referral Centre for Rare Autoimmune and Systemic Diseases, Hôp. Cochin, AP-HP; Université Paris Descartes, Paris; and Dept.of Internal Medicine and Clinical Immunology, François-Mitterrand Hosp., Dijon University Hosp., France.
  2. Department of Internal Medicine, Referral Centre for Rare Autoimmune and Systemic Diseases, Hôpital Cochin, AP‒HP; Université Paris Descartes, Paris, France.
  3. Department of Internal Medicine, Referral Centre for Rare Autoimmune and Systemic Diseases, Hôpital Cochin, AP‒HP; Université Paris Descartes, Paris, France.
  4. Department of Internal Medicine and Systemic Diseases, François-Mitterrand Hospital, Dijon University Hospital, Dijon, France.
  5. Department of Internal Medicine, Referral Centre for Rare Autoimmune and Systemic Diseases, Hôpital Cochin, AP‒HP; Université Paris Descartes, Paris, France.
  6. Department of Internal Medicine, CHU Côte de Nacre, Caen, France.
  7. Department of Internal Medicine, CHU Dupuytren, Limoges, France.
  8. Department of Nephrology and Internal Medicine, CH Poissy, Saint-Germain-en-Laye, France.
  9. Department of Nephrology and Internal Medicine, CH de Verdun, France.
  10. Department of Internal Medicine, CHU de Clermont-Ferrand, France.
  11. Department of Rheumatology, CH René-Dubos, Pontoise, France.
  12. Department of Internal Medicine, CHU de Nantes, France.
  13. Division of Rheumatology, Mount Sinai Hospital, University Health Network, University of Toronto, Canada.
  14. Department of Internal Medicine, Referral Centre for Rare Autoimmune and Systemic Diseases, Hôpital Cochin, AP‒HP; Université Paris Descartes, Paris, France.
  15. Department of Internal Medicine, Referral Centre for Rare Autoimmune and Systemic Diseases, Hôpital Cochin, AP‒HP; Université Paris Descartes, Paris, France.

for the French Vasculitis Study Group (FVSG)

CER10441
2017 Vol.35, N°1 ,Suppl.103
PI 0176, PF 0184
Treatment

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PMID: 28422001 [PubMed]

Received: 23/03/2017
Accepted : 28/03/2017
In Press: 18/04/2017
Published: 20/04/2017

Abstract

OBJECTIVES:
To analyse the 10-year outcomes of 64 patients with non-HBV polyarteritis nodosa (PAN) or microscopic polyangiitis (MPA) and Five-Factor Score-defined poor-prognosis factors enrolled (1994-2000) in the prospective, randomised, open-label CHUSPAN trial.
METHODS:
The 64 patients were randomised to receive 12 (33: 23 MPA, 10 PAN) or 6 (31: 17 MPA, 14 PAN) cyclophosphamide (CYC) pulses combined with glucocorticoids. Ten-year follow-up of these patients included times to relapse(s), failure(s) and/or deaths calculated from treatment onset. Data were censored after 120 months of follow-up.
RESULTS:
Eleven patients were lost to-follow-up (mean±SD follow-up: 61.9±35.2 months), with no between-group difference. As previously reported, baseline clinical characteristics and laboratory values were comparable for the 2 groups. After induction, 53/64 (83%) entered remission, with comparable percentages for both groups. The regimen was intensified for 11 initial non-responders: 4 achieved remission and 8 died before doing so. During extended follow-up, 26 patients experienced ≥1 relapse(s): 12 in the 12-pulse group and 14 in the 6-pulse group (p=0.47). At 10 years, overall and disease-free survival rates were 57.4% and 29.9%, with no between-group differences (p=0.185 and p=0.367, respectively). Factors associated with shorter disease-free survival were age ≥65 years and alveolar haemorrhage at diagnosis.
CONCLUSIONS:
Although the 3-year CHUSPAN trial results indicated the superiority of 12 vs. 6 CYC pulses, that early advantage progressively declined and became non-significant by 10 years.

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