Journal Article

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

Arsène Mekinian, Eric Grignano, Thorsten Braun, Olivier Decaux, Eric Liozon, Nathalie Costedoat-Chalumeau, Jean-Emmanuel Kahn, Mohammed Hamidou, Sophie Park, Xavier Puéchal, Eric Toussirot, Géraldine Falgarone, David Launay, Nathalie Morel, Sébastien Trouiller, Alexis Mathian, Bruno Gombert, Yoland Schoindre, Bertrand Lioger, Benoit De Wazieres, Zahir Amoura, Anne-Laure Buchdaul, Sophie Georgin-Lavialle, Jérémie Dion, Serge Madaule, Loïc Raffray, Pascal Cathebras, Jean Charles Piette, Christian Rose, Jean Marc Ziza, Olivier Lortholary, Francois Montestruc, Mohammed Omouri, Guillaume Denis, Julien Rossignol, Stanislas Nimubona, Lionel Adès, Claude Gardin, Pierre Fenaux and Olivier Fain

in Rheumatology

Volume 55, issue 2, pages 291-300
Published in print February 2016 | ISSN: 1462-0324
Published online September 2015 | e-ISSN: 1462-0332 | DOI: https://dx.doi.org/10.1093/rheumatology/kev294
Systemic inflammatory and autoimmune manifestations associated with myelodysplastic syndromes and chronic myelomonocytic leukaemia: a French multicentre retrospective study

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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: myelodysplastic syndrome; autoimmune disorders; treatment; outcome

Journal Article.  5657 words.  Illustrated.

Subjects: Rheumatology ; Immunology

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