Dermatomyosites (DM) à anticorps anti-Mi2 revisitées : DM pure avec nécrose musculaire et haut risque de malignité associée

International audienc

Anti-Mi2 dermatomyositis revisited: pure DM phenotype with muscle fiber necrosis and high risk of malignancy

IF 2.969International audienceAnti-Mi2 are dermatomyositis-specific autoantibodies (Aabs) that have been associated, in small cohorts, with a good prognosis possibly related to a lower malignancy risk. Our objective was to describe the phenotype of anti-Mi-2 dermatomyositis (DM) in a larger cohort. A national multicenter retrospective cohort study was performed including all patients with a clinical phenotype suggestive of DM (cutaneous manifestations and/or muscle involvement) and a positive anti-Mi2 Aabs. Medical records were retrospectively reviewed to assess clinical and histological features, and presence of cancer occurring ± 3 years of diagnosing myositis (CAM)

Performances de l’IRM musculaire pour le diagnostic des myopathies auto-immunes : étude prospective multicentrique (DARWIM)

International audienc

Efficacy of anti-TNF alpha in severe and/or refractory Beh\ue7et's disease: Multicenter study of 124 patients

Objective: To report the efficacy and safety of anti-TNF agents in patients with severe and/or refractory manifestations of Beh\ue7et's disease (BD). Methods: We performed a multicenter study of main characteristics and outcomes of anti-TNF alpha treatments [mainly infliximab (62%), and adalimumab (30%)] in 124 BD patients [48% of men; median age of 33.5 (28-40) years]. Results: Overall response (i.e. complete and partial) rate was 90.4%. Clinical responses were observed in 96.3%, 88%, 70%, 77.8%, 92.3% and 66.7% of patients with severe and/or refractory ocular, mucocutaneous, joint, gastro-intestinal manifestations, central nervous system manifestations and cardiovascular manifestations, respectively. No significant difference was found with respect to the efficacy of anti-TNF used as monotherapy or in association with an immunosuppressive agent. The incidence of BD flares/patient/year was significantly lower during anti-TNF treatment (0.2\ub10.5 vs 1.7\ub12.4 before the use of anti-TNF, p<0.0001). The prednisone dose was significantly reduced at 6 and 12 months (p<0.0001). In multivariate analysis, retinal vasculitis was negatively associated with complete response to anti-TNF (OR=0.33 [0.12-0.89]; p=0.03). The efficacy and relapse free survival were similar regardless of the type of anti-TNF agent used. After a median follow-up of 21 [7-36] months, side effects were reported in 28% of patients, including infections (16.3%) and hypersensitivity reactions (4.1%). Serious adverse events were reported in 13% of cases. Conclusion: Anti-TNF alpha therapy is efficient in all severe and refractory BD manifestations. Efficacy appears to be similar regardless of the anti-TNF agent used (infliximab or adalimumab)

Vitamin D and autoimmune diseases

Vitamin D and its deficiency are becoming a subject of great interest in recent years. In addition to the well-known role of vitamin D in maintaining bone health, evidence from recent years are accumulating in favor of its importance in the functioning of the immune system. The association between vitamin D deficiency and autoimmune diseases has been supported by epidemiological studies, demonstrating higher prevalence of vitamin D deficiency among autoimmune patients, in comparison to the general population. Vitamin D was also associated to various autoimmune diseases in both molecular and interventional studies; among the associated diseases are: systemic lupus erythematosus, type 1 diabetes mellitus, multiple sclerosis and others. In this review, relevant literature on the association between autoimmunity and vitamin D deficiency will be reviewed and discussed, as well as a summary of important recommendations for vitamin D supplementations in autoimmune patients