Characteristics and Management of IgA Vasculitis (Henoch-Schonlein) in Adults Data From 260 Patients Included in a French Multicenter Retrospective Survey - Aix-Marseille Université Access content directly
Journal Articles Arthritis & rheumatology Year : 2017

Characteristics and Management of IgA Vasculitis (Henoch-Schonlein) in Adults Data From 260 Patients Included in a French Multicenter Retrospective Survey

1 Hôpital Cochin [AP-HP]
2 I3 - Immunologie - Immunopathologie - Immunothérapeutique
3 Centre d'Epidémiologie Clinique
4 IC UM3 (UMR 8104 / U1016) - Institut Cochin
5 CHU Pitié-Salpêtrière [AP-HP]
6 Centre hospitalier [Valenciennes, Nord]
7 I3 - Immunologie - Immunopathologie - Immunothérapie
8 AMU - Aix Marseille Université
9 VRCM - Vascular research center of Marseille
10 CHU Angers - Centre Hospitalier Universitaire d'Angers
11 CHU Toulouse - Centre Hospitalier Universitaire de Toulouse
12 PIMIT - Processus Infectieux en Milieu Insulaire Tropical
13 Clinique de médecine interne
14 Hôpital Necker - Enfants Malades [AP-HP]
15 Service de Médecine Interne
16 CHU de Poitiers [La Milétrie] - Centre hospitalier universitaire de Poitiers = Poitiers University Hospital
17 Département de Néphrologie et Transplantation d'organes [CHU Toulouse]
18 CHU ST-E - Centre Hospitalier Universitaire de Saint-Etienne [CHU Saint-Etienne]
19 UNS UFR Médecine - Université Nice Sophia Antipolis - Faculté de Médecine
20 Service de médecine interne
21 UNINE - Université de Neuchâtel = University of Neuchatel
22 CHU de Lille - Centre Hospitalier Universitaire de Lille
23 CHU Caen
24 CHRU Tours - Centre Hospitalier Régional Universitaire de Tours
25 NORT - Nutrition, obésité et risque thrombotique
26 CHU Saint-Antoine [AP-HP]
27 UFR des Sciences et Technologies
28 Service de médecine interne et centre de référence des maladies rares [CHU Cochin]
29 Service de néphrologie adultes [CHU Necker]
30 Service de rhumatologie
Agnès Dechartres
Johan Chanal
  • Function : Author
Alban Deroux
  • Function : Author
  • PersonId : 964950
Aurélie Baldolli
  • Function : Author
Xavier Puéchal
Boris Bienvenu
  • Function : Author
Arsene Mekinian
Elie Zagdoun
  • Function : Author


Methods: Data on clinical characteristics, histologic features, and treatment response from 260 patients with IgAV included in a French multicenter retrospective survey were analyzed. Efficacy data were compared using different statistical models. Results: The mean ± SD age of the patients with IgAV at diagnosis was 50.1 ± 18 years, and 63% of patients were male. Baseline manifestations included purpura (100%), arthralgias/arthritis/myalgia (61%), glomerulonephritis (70%), and/or gastrointestinal involvement (53%). Thirty percent of patients showed renal failure at baseline. In univariate analysis, the response to therapy was 80% (64 of 80) in patients treated with corticosteroids (CS) alone, compared to 77% (23 of 30) in patients treated with CS plus cyclophosphamide (CYC) and 59% (10 of 17) in patients treated with colchicine (P = 0.17). Multivariable analysis showed that treatment with CS or CS plus CYC was more effective than colchicine in achieving a response. Efficacy differences were demonstrated using different statistical models: in the multivariable logistic regression model, odds ratio (OR) 3.68, 95% confidence interval (95% CI) 1.10–12.33 (P = 0.03); in the inverse probability weighting on propensity score model, OR 3.75, 95% CI 1.28–10.99 (P = 0.02). The efficacy of CS plus CYC as compared to CS alone was discordant according to the analytic method used. Analysis with the multivariable logistic regression model did not demonstrate a difference between CS plus CYC and CS alone (OR 0.88, 95% CI 0.29–2.67; P = 0.82). In contrast, inverse probability weighting on propensity score showed that CS plus CYC was more effective than CS alone (OR 1.79, 95% CI 1.00–3.20; P = 0.049). Conclusion: This series constitutes the largest series of adults with IgAV reported in the literature so far. It provides data on clinical and histologic presentation and therapeutic efficacy, suggesting that CS alone appears to be a reasonable first‐line therapy in patients with IgAV, while the benefit of adding CYC to CS remains uncertain.
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hal-01787239 , version 1 (14-05-2018)



Alexandra Audemard-Verger, Benjamin Terrier, Agnès Dechartres, Johan Chanal, Zahir Amoura, et al.. Characteristics and Management of IgA Vasculitis (Henoch-Schonlein) in Adults Data From 260 Patients Included in a French Multicenter Retrospective Survey. Arthritis & rheumatology, 2017, 69 (9), pp.1862-1870. ⟨10.1002/art.40178⟩. ⟨hal-01787239⟩
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