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Multicenter Study
. 2016 Feb;55(2):291-300.
doi: 10.1093/rheumatology/kev294. Epub 2015 Sep 8.

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

Arsène Mekinian  1 , Eric Grignano  1 , Thorsten Braun  2 , Olivier Decaux  3 , Eric Liozon  4 , Nathalie Costedoat-Chalumeau  5 , Jean-Emmanuel Kahn  6 , Mohammed Hamidou  7 , Sophie Park  8 , Xavier Puéchal  5 , Eric Toussirot  9 , Géraldine Falgarone  10 , David Launay  11 , Nathalie Morel  5 , Sébastien Trouiller  12 , Alexis Mathian  13 , Bruno Gombert  14 , Yoland Schoindre  6 , Bertrand Lioger  15 , Benoit De Wazieres  16 , Zahir Amoura  13 , Anne-Laure Buchdaul  17 , Sophie Georgin-Lavialle  18 , Jérémie Dion  5 , Serge Madaule  19 , Loïc Raffray  20 , Pascal Cathebras  21 , Jean Charles Piette  5 , Christian Rose  22 , Jean Marc Ziza  23 , Olivier Lortholary  24 , Francois Montestruc  25 , Mohammed Omouri  26 , Guillaume Denis  27 , Julien Rossignol  28 , Stanislas Nimubona  29 , Lionel Adès  30 , Claude Gardin  2 , Pierre Fenaux  30 , Olivier Fain  31
Affiliations
Multicenter Study

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

Arsène Mekinian et al. Rheumatology (Oxford). 2016 Feb.

Abstract

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 < 0.01), male (P = 0.03), less often had refractory anaemia with ring sideroblasts (P < 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.

Keywords: autoimmune disorders; myelodysplastic syndrome; outcome; treatment.

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