Systemic inflammatory and autoimmune manifestations associated with myelodysplastic syndromes and chronic myelomonocytic leukaemia: a French multicentre retrospective study
Systemic inflammatory and autoimmune manifestations associated with myelodysplastic syndromes and chronic myelomonocytic leukaemia: a French multicentre retrospective study
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DOI：
10.1093/rheumatology/kev294
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发表时间：
2016-02-01
期刊：
影响因子：
5.5
通讯作者：
Fain, Olivier
中科院分区：
文献类型：
--
作者：
Mekinian, Arsene;Grignano, Eric;Fain, Olivier
目标。我们在法国的一项多中心回顾性研究中描述了骨髓增生异常综合征（MDS）相关的系统性炎症和自身免疫性疾病（SIADs），它们的治疗和结局以及SIADs对总生存率的影响。本研究对123例MDS合并siad患者进行了分析。平均年龄70岁（sd = 13），男女比例为2。siad为全身性血管炎39例（32%），CTD 31例（25%），炎性关节炎28例（23%），中性粒细胞紊乱12例（10%），未分类13例（11%）。75例（66%）siad符合通常的分类标准，21例（19%）siad未达到完全的分类标准。慢性髓细胞白血病（CMML）与全身性血管炎有显著相关性（P = 0.0024）。118例（96%）SIAD患者接受了治疗（91%使用类固醇），其中83%对一线治疗有反应，其中80%单独使用类固醇。48%的siad患者需要接受类固醇依赖或复发的二线治疗。11例阿扎胞苷治疗的患者可以评估MDS治疗SIAD的效果，分别在3个月和6个月时，9/11（80%）和6/11（55%）患者实现了SIAD缓解。与665例不伴有SIADs的MDS/CMML患者相比，伴有SIADs的MDS/CMML患者更年轻（P< 0.01），男性（P = 0.03），伴有环状铁母细胞的难治性贫血较少（P< 0.01），核型较差的患者较多（16% vs 11%, P = 0.04），属于国际预后评分系统低、中1级的患者较少，但伴有MDS相关SIADs的患者与不伴有SIADs的患者生存率无差异（P = 0.5）。与MDS相关的siad是异质性的，类固醇敏感的，但通常是类固醇依赖的。
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.