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

Arsene Mekinian 1, 2, * Guillaume Dervin 2, 1 Nathanael Lapidus 3 Jean-Emmanuel Kahn 4 Louis Terriou 5 Eric Liozon 6 Eric Grignano 2 Jean-Charles Piette 7 Odile Beyne Rauzy 8 Vincent Grobost 9 Pascal Godmer 10 Jerome Gillard 11 Julien Rossignol 12 David Launay 5 Achille Aouba 13 Thierry Cardon 14 Laurence Bouillet 15 Jonathan Broner 16 Julien Vinit 17 Lionel Ades 18 Fabrice Carrat 3 Clementine Salvado 19 Eric Toussirot 20 Mathilde Versini 21 Nathalie Costedoat-Chalumeau 22, 23, 24 Jean Baptiste Fraison 22, 23, 24 Philippe Guilpain 16 Pierre Fenaux 18 Olivier Fain 2, 1
Abstract : Background: 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.
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Autoimmunity Reviews, Elsevier, 2017, 〈10.1016/j.autrev.2017.07.003〉
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Arsene Mekinian, Guillaume Dervin, Nathanael Lapidus, Jean-Emmanuel Kahn, Louis Terriou, et al.. Biologics in myelodysplastic syndrome-related systemic inflammatory and autoimmune diseases: French multicenter retrospective study of 29 patients. Autoimmunity Reviews, Elsevier, 2017, 〈10.1016/j.autrev.2017.07.003〉. 〈hal-01563947〉

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