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