Autoimmune diseases in myelodysplastic syndrome favors patients survival: A case control study and literature review.

Abstract Background 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. Results 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)]. Conclusion 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. Highlights • Autoimmune and inflammatory diseases associated with MDS are heterogeneous. • Autoimmune and inflammatory diseases are not associated to a specific MDS subtype. • MDS patients with Autoimmune and inflammatory diseases seems to have a better overall survival than other MDS patients. [ABSTRACT FROM AUTHOR]