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Article Dans Une Revue Rheumatology Année : 2016

Systemic inflammatory and autoimmune manifestations associated with myelodysplastic syndromes and chronic myelomonocytic leukaemia: a French multicentre retrospective study

Arsène Mekinian
Thorsten Braun
  • Fonction : Auteur
Mohammed Hamidou
  • Fonction : Auteur
Xavier Puéchal
Nathalie Morel
  • Fonction : Auteur
Sébastien Trouiller
  • Fonction : Auteur
Bruno Gombert
  • Fonction : Auteur
Yoland Schoindre
  • Fonction : Auteur
  • PersonId : 934581
Benoit De Wazieres
  • Fonction : Auteur
Anne-Laure Buchdaul
  • Fonction : Auteur
Sophie Georgin-Lavialle
Jérémie Dion
  • Fonction : Auteur
Serge Madaule
  • Fonction : Auteur
Loïc Raffray
  • Fonction : Auteur
  • PersonId : 992887
Jean Charles Piette
  • Fonction : Auteur
Jean Marc Ziza
  • Fonction : Auteur
Francois Montestruc
  • Fonction : Auteur
Mohammed Omouri
  • Fonction : Auteur
Guillaume Denis
  • Fonction : Auteur
Julien Rossignol
  • Fonction : Auteur
Lionel Adès
Pierre Fenaux
  • Fonction : Auteur
Olivier Fain

Résumé

Objective. We describe myelodysplastic syndrome (MDS)–associated systemic inflammatory and autoimmune diseases (SIADs), their treatments and outcomes and the impact of SIADs on overall survival in a French multicentre retrospective study. Methods. In this study, 123 patients with MDS and SIADs were analysed. Results. Mean age was 70 years (s.d. 13) and the male:female ratio was 2. The SIADs were systemic vasculitis in 39 (32%) cases, CTD in 31 (25%) cases, inflammatory arthritis in 28 (23%) cases, a neutrophilic disorder in 12 (10%) cases and unclassified in 13 cases (11%). The SIADs fulfilled the usual classification criteria in 75 (66%) cases, while complete criteria were not reached in 21 (19%) cases. A significant association was shown between chronic myelomonocytic leukaemia (CMML) and systemic vasculitis (P = 0.0024). One hundred and eighteen (96%) SIAD patients were treated (91% with steroids), with an 83% response to first-line treatment, including 80% for steroids alone. A second-line treatment for SIADs was required for steroid dependence or relapse in 48% of cases. The effect of MDS treatment on SIADs could be assessed in 11 patients treated with azacytidine and SIAD response was achieved in 9/11 (80%) and 6/11 (55%) patients at 3 and 6 months, respectively. Compared with 665 MDS/CMML patients without SIADs, MDS/CMML patients with SIADs were younger (P \textless 0.01), male (P = 0.03), less often had refractory anaemia with ring sideroblasts (P \textless 0.01), more often had a poor karyotype (16% vs 11%, P = 0.04) and less frequently belonged to low and intermediate-1 International Prognostic Scoring System categories, but no survival difference was seen between patients with MDS-associated SIADs and without SIADs (P = 0.5). Conclusion. The spectrum of SIADs associated to MDS is heterogeneous, steroid sensitive, but often steroid dependent

Dates et versions

hal-01299293 , version 1 (07-04-2016)

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Arsène Mekinian, Eric Grignano, Thorsten Braun, Olivier Decaux, Eric Liozon, et al.. Systemic inflammatory and autoimmune manifestations associated with myelodysplastic syndromes and chronic myelomonocytic leukaemia: a French multicentre retrospective study. Rheumatology, 2016, 55 (2), pp.291--300. ⟨10.1093/rheumatology/kev294⟩. ⟨hal-01299293⟩
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