Aix-Marseille Univ, APHM, Medecine Interne Hôpital de la Timone, Marseille, France.
Aix-Marseille Univ, Inserm, CNRS, Institut Paoli-Calmettes, CRCM, Marseille, France.
Autoimmun Rev. 2019 Jan;18(1):36-42. doi: 10.1016/j.autrev.2018.07.009. Epub 2018 Nov 5.
We conducted a monocentric retrospective study of patients with myelodysplastic syndromes (MDS) and autoimmune or inflammatory disorders (AIMs) and a literature review. We analyzed the association with subgroups of the WHO 2016 MDS classification and patient's survival in a case control study. Risk factors associated with survival were analyzed by uni- and multivariate analysis.
From all MDS patients 11% presented with AIMs. These were heterogeneous and the most frequent where polyarthritis (25%) and autoimmune cytopenias (17%). No difference for frequency and type of AIMs was observed for the WHO 2016 MDS subgroups (p = .3). In the case control study WHO classification, karyotype abnormalities, IPSS-R and IPSS were similar in both groups. The overall survival from MDS diagnosis was better in the group with AIMs [10.3 ± 0.6 (IC95% 6.2-12.9) versus 4.8 ± 1.1 years (IC95% 4.2-8.7), p = .04]. The better survival was restricted to MDS with low or intermediate-1 IPSS [11.1 ± 1.5 (IC95% 9.9-NR) versus 8.7 ± 1.3 years (IC95% 4.8-10.3), p = .006]. The better survival was only observed when AIMs diagnosis was timely associated or appeared after MDS diagnosis (p = .04). Factors associated with a better overall survival and survival without AML were steroid dependence [respectively HR = 0.042, p = .003, (IC95% 0.005-0.33) and HR = 0.07, p = .002, (IC95% 0.013-0.39)], a diagnosis of AIMs and MDS timely associated [respectively HR = 0.05, p = .009, (IC95% 0.006-0.478) and HR = 0.1, p = .008, (IC95% 0.018-0.54)] or a diagnosis of AIMs after MDS [respectively HR = 0.024, p = .009, (IC95% 0.001-0.39) and HR = 0.04, p = .008, (IC95% 0.003-0.43)].
Autoimmune and inflammatory diseases associated to MDS are heterogeneous. AIMs diagnosed after or concomitantly to MDS seems associated with a better survival. Prospective studies are necessary to demonstrate that autoimmunity is associated to a better control of the MDS clone.
我们进行了一项单中心回顾性研究，纳入了伴自身免疫或炎症性疾病（AIMs）的骨髓增生异常综合征（MDS）患者，并进行了文献复习。我们分析了与 2016 年 WHO MDS 分类亚组的相关性，并在病例对照研究中分析了患者的生存情况。通过单因素和多因素分析，分析了与生存相关的危险因素。
所有 MDS 患者中，11%伴有 AIMs。这些疾病具有异质性，最常见的是多发性关节炎（25%）和自身免疫性血细胞减少症（17%）。对于 2016 年 WHO MDS 亚组，AIMs 的频率和类型无差异（p=0.3）。在病例对照研究中，WHO 分类、核型异常、IPSS-R 和 IPSS 在两组间相似。伴有 AIMs 的 MDS 患者从 MDS 诊断后的总体生存时间更好[10.3±0.6（95%CI 6.2-12.9）与 4.8±1.1 年（95%CI 4.2-8.7），p=0.04]。这种更好的生存仅限于低危或中危-1 的 IPSS MDS[11.1±1.5（95%CI 9.9-NR）与 8.7±1.3 年（95%CI 4.8-10.3），p=0.006]。只有当 AIMs 的诊断及时相关或在 MDS 诊断后出现时，更好的生存才会被观察到（p=0.04）。与总体生存和无 AML 生存相关的因素包括激素依赖[分别为 HR=0.042，p=0.003（95%CI 0.005-0.33）和 HR=0.07，p=0.002（95%CI 0.013-0.39）]、及时相关的 MDS 和 AIMs 诊断[分别为 HR=0.05，p=0.009（95%CI 0.006-0.478）和 HR=0.1，p=0.008（95%CI 0.018-0.54）]或 MDS 后诊断的 AIMs[分别为 HR=0.024，p=0.009（95%CI 0.001-0.39）和 HR=0.04，p=0.008（95%CI 0.003-0.43）]。
伴 MDS 的自身免疫和炎症性疾病具有异质性。在 MDS 后或同时诊断的 AIMs 似乎与更好的生存相关。需要前瞻性研究来证明自身免疫与 MDS 克隆的更好控制有关。
