Analyse des stratégies thérapeutiques dans la maladie de Behçet (étude retrospective monocentrique de 98 patients)

La maladie de Behçet est une vascularite qui expose à des complications sévères. Nous avons mené une étude rétrospective pour décrire les patients atteints de la maladie de Behçet satisfaisant les critères ICBD 2013 et les stratégies thérapeutiques qui leur sont proposées. L'étude a inclus 98 patients suivis entre 2000 et 2012 au CHU de Montpellier. Il s'agissait de 56 hommes et 42 femmes sex ratio 1,33. L'âge moyen est de 42,3 ans. L'âge moyen des premières manifestations était de 22.6 ans et le retard diagnostic moyen était de 10,2 ans. L' origine des patients était à 50% européenne. Les principales manifestations étaient : aphtose orale 100%, aphtose génitale 67,3%. uvéite 68,4% (surtout postérieure ou totale 78%, cécité 13%), atteinte articulaire 59,2%, neurologiques 58,2% dont 27 patients (51 ,9%) avec atteintes psychiatriques, vasculaire 34%, digestive 33%. cardiaque 7,1 %, testiculaire 23% des hommes et pulmonaire 4,1%. Une prédominance masculine était trouvée pour l'atteinte ophtalmologique (OR à 2,43) et féminine pour l'atteinte articulaire (OR à 0,33). Les traitements sont décrits ainsi que leur tolérance. Vingt et un patients ont été traités par anti-TNF a Les anti-TNF a ont permis de baisser significativement le seuil de cortico-dépendance des patients réfractaires au moins 1 autre immunosuppresseur et de diminuer le nombre de poussées de la maladie. Sous anti-TNF a. l'acuité visuelle augmentait significativement (p=0.001). Dans la discussion, le protil clinique de notre série est comparé à ceux de la littérature. Les données thérapeutiques actuellement publiées sont présentées. La place des anti-TNF a dans le schéma thérapeutique est discutéeMONTPELLIER-BU Médecine UPM (341722108) / SudocMONTPELLIER-BU Médecine (341722104) / SudocSudocFranceF

Inflammatory disorders associated with trisomy 8‐myelodysplastic syndromes: French retrospective case‐control study

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Efficacy of Azacitidine (AZA) in Autoimmune and Inflammatory Disorders (AID) Associated with Myelodysplastic Syndromes (MDS) and Chronic Myelomonocytic Leukemia (CMML)

doublon?International audience57th Annual Meeting of the American-Society-of-Hematology Location: Orlando, FL Date: DEC 05-08, 201

Efficacy of Azacitidine in Autoimmune and Inflammatory Disorders associated with Myelodysplastic Syndromes and Chronic Myelomonocytic Leukemia

International audienceThis retrospective study describes efficacy of Azacitidine on autoimmune disorders (AID) associated with MDS/CMML in 22 patients. Response of AID to Azacitidine was observed in 19 patients (86%). Reduction or discontinuation of steroids and/or immunosuppressive therapy (IST) was possible in 16 cases (73%). Hematologic response was seen in 55% of the patients. MDS/CMML and AID evolution was concordant in 13 cases (59%): both favorable (n = 11), both unfavorable (n = 2), but AID improved while MDS/CMML worsened (n = 8) and vice versa (n = 1). Azacitidine frequently seems effective in controlling steroid-dependent AID associated with MDS/CMML, but prospective studies are necessary to confirm those finding

Inflammatory Disorders Associated with Trisomy 8-myelodysplastic Syndromes: French Retrospective Case-control Study

International audienc

Gastrointestinal Behcet's-like Disease with Myelodysplastic Neoplasms with Trisomy 8: A French Case Series and Literature Review

International audienc

Biologics in myelodysplastic syndrome-related systemic inflammatory and autoimmune diseases: French multicenter retrospective study of 29 patients

International audienceBackground: Systemic inflammatory and autoimmune diseases (SIADs) associated with myelodysplastic syndromes are often difficult to treat. Corticosteroids are efficient but only usually at high doses. The use of biologics needs to be specified.Methods: In a French multicenter retrospective study, we analyzed the efficacy and safety of biologics (tumor necrosis factor-α [TNF-α] antagonists, tocilizumab, rituximab and anakinra) for SIADs associated with myelodysplastic syndromes (MDSs). Clinical, biological and overall treatment responses were evaluated. When several lines of treatment were used, data were analyzed before and at the end of each treatment line and were pooled to compare overall response among steroids, disease-modifying anti-rheumatic drugs (DMARDs) and biologics.Results: We included 29 patients (median age 67 years [interquartile range 62–76], 83% males) with MDS-related SIADs treated with at least one biologic. The MDSs were predominantly refractory anemia with excess blasts 1 (38%) and refractory cytopenia with multilineage dysplasia (21%). The SIADs were mainly arthritis (n = 6; 20%), relapsing polychondritis (n = 8; 30%) and vasculitis (n = 10; 34%). During a 3-year median follow-up (IQR 1.3–4.5), a total of 114 lines of treatments were used for all patients: steroids alone (22%), DMARDs (23%), TNF-α antagonists (14%), anakinra (10%), rituximab (10%), tocilizumab (7%) and azacytidine (9%). Considering all 114 lines, overall response (complete and partial) was shown in 54% cases. Overall response was more frequent with steroids (78%) and rituximab (66%) than DMARDs (45%) and other biologics (33%) (p < 0.05). Rituximab had better response in vasculitis and TNF-α antagonists in arthritis. During follow-up, 20 patients (71%) presented at least one severe infection.Conclusion: This nationwide study demonstrates the efficacy of steroids for SIAD-associated MDSs but a high frequency of steroid dependence. The response to biologics seems low, but rituximab and azacytidine seem promising

Infliximab versus adalimumab in the treatment of refractory inflammatory uveitis: Multicenter study from the french uveitis network

International audienceOBJECTIVES: To analyze the factors associated with response to anti-TNFα and to compare the efficacy and safety of infliximab (IFX) and adalimumab (ADA) in patients with refractory non infectious uveitis.METHODS: Observational multicenter study including 160 patients [median age of 31 [21-42] years with 39% of men] with refractory uveitis treated with anti-TNFα [IFX 5mg/kg at weeks 0, 2, 6 and every 5-6 weeks (n=98) and ADA 40mg/14days (n=62)]. Factors associated to complete response were assessed in multivariate analysis. Comparison between efficacy and safety of IFX and ADA was performed using a propensity score approach accounting for baseline characteristics.RESULTS: Main etiologies of uveitis included Behçet's disease (36%), juvenile idiopathic arthritis (22%), spondylarthropathies (10%) and sarcoidosis (6%). The overall response rate at 6 and 12 months was of 87% (26% of complete response) and 93% (28% of complete response), respectively. The median time to complete response was 2 (0-12) months. In multivariate analysis, Behçet's disease (SHR= 2.52 [1.35-7.71], p=0.004) and a number of uveitis flares before anti-TNFα greater than 5 (HR=1.97 [1.02-3.84], p=0.045) were associated with complete response to anti-TNFα. Side effects were reported in 28% of patients, including 12% of serious adverse events. IFX and ADA did not differ significantly in terms of occurrence of complete response (SHR=0.65 [0.25;1.71], p=0.39), serious side effects (SHR= 0.22 [0.04-1.25], p=0.089) or event free survival (SHR=0.55 [0.28;1.08], p=0.083).CONCLUSIONS: Anti-TNFα are highly effective in refractory inflammatory uveitis. Behçet's disease is associated with increase odds of response. IFX and ADA seem equivalent in terms of efficacy. This article is protected by copyright. All rights reserve