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

Autor:	Samson M; 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., Puéchal X; Department of Internal Medicine, Referral Centre for Rare Autoimmune and Systemic Diseases, Hôpital Cochin, AP‒HP; Université Paris Descartes, Paris, France., Mouthon L; Department of Internal Medicine, Referral Centre for Rare Autoimmune and Systemic Diseases, Hôpital Cochin, AP‒HP; Université Paris Descartes, Paris, France., Devilliers H; Department of Internal Medicine and Systemic Diseases, François-Mitterrand Hospital, Dijon University Hospital, Dijon, France., Cohen P; Department of Internal Medicine, Referral Centre for Rare Autoimmune and Systemic Diseases, Hôpital Cochin, AP‒HP; Université Paris Descartes, Paris, France., Bienvenu B; Department of Internal Medicine, CHU Côte de Nacre, Caen, France., Ly KH; Department of Internal Medicine, CHU Dupuytren, Limoges, France., Bruet A; Department of Nephrology and Internal Medicine, CH Poissy, Saint-Germain-en-Laye, France., Gilson B; Department of Nephrology and Internal Medicine, CH de Verdun, France., Ruivard M; Department of Internal Medicine, CHU de Clermont-Ferrand, France., Pertuiset E; Department of Rheumatology, CH René-Dubos, Pontoise, France., Hamidou M; Department of Internal Medicine, CHU de Nantes, France., Pagnoux C; Division of Rheumatology, Mount Sinai Hospital, University Health Network, University of Toronto, Canada., Terrier B; Department of Internal Medicine, Referral Centre for Rare Autoimmune and Systemic Diseases, Hôpital Cochin, AP‒HP; Université Paris Descartes, Paris, France., Guillevin L; Department of Internal Medicine, Referral Centre for Rare Autoimmune and Systemic Diseases, Hôpital Cochin, AP‒HP; Université Paris Descartes, Paris, France.
Jazyk:	angličtina
Zdroj:	Clinical and experimental rheumatology [Clin Exp Rheumatol] 2017 Mar-Apr; Vol. 35 Suppl 103 (1), pp. 176-184. Date of Electronic Publication: 2017 Apr 18.
Abstrakt:	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.
Databáze:	MEDLINE
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