现有的自身免疫性疾病会影响10-30%的骨髓增殖异常综合征（MDS）患者。有关患有和不患有自身免疫性疾病的MDS患者结局的研究结果不一致。为了确定自免疫对MDS结局的影响，我们利用监测、流行病学和结果（SEER-Medicare）数据库进行了人群分析。病例数据为2007年至2017年，利用索赔算法识别自身免疫性疾病、人口特征、共病评分、MDS组织学、输血负担、低甲基化剂治疗以及造血干细胞移植情况。Cox回归模型评估了对生存的影响，竞争风险回归模型定义了对白血病转化的影响。我们分析了15,277例MDS患者，其中2,442例（16%）有既往自身免疫性疾病。具有既往自免疫性的病例具有独特的流行病学特点，他们年龄较小，以女性为主，输血负担较高，但MDS组织学分布无差异。自身免疫性疾病与11%的死亡风险降低相关（风险比[HR] 0.89，95%可信区间[CI] 0.85 - 0.94, p<0.001）。对于白血病转化风险的影响因MDS组织学而异。在低风险MDS组织学中，免疫性疾病与白血病的风险增加1.9倍（HR 1.87，95%CI 1.17 - 2.99, p=0.008），而其他组中没有显著效果。这些结果表明自身免疫性疾病影响MDS的生存，并与降低的死亡率相关。尽管具有更高白血病进展的风险，但在低风险组织学中仍明显见到了生存效应。这可能代表了同时有利于较不侵袭性表型和克隆扩张的炎症驱动造血过程，这需要进一步研究。 © 2023 美国血液学会。

Preexisting autoimmune disease affects 10-30% of patients with myelodysplastic syndromes (MDS). Studies comparing outcomes in MDS patients with and without autoimmune disease show discordant results. Using Surveillance, Epidemiology, and End Results (SEER)-Medicare, we conducted a population analysis to define the impact of autoimmunity on MDS outcomes. Cases were ascertained between 2007 - 2017 and claim algorithms employed to identify autoimmune disease, demographic characteristics, comorbidity scores, MDS histology, transfusion burden, treatment with hypomethylating agents and hematopoietic stem cell transplant. Cox regression models estimated the impact on survival and competing-risk regression models defined the effect on leukemic transformation. We analyzed 15,277 MDS patients, including 2,442 (16%) with preexisting autoimmune disease. The epidemiologic profile was distinctive in cases with preexisting autoimmunity, who were younger, predominantly female and had higher transfusion burden without difference in MDS histologic distribution. Autoimmune disease was associated with 11% decreased risk of death (hazard ratio [HR] 0.89, 95% confidence interval [CI], 0.85 - 0.94, p<0.001). The effect on risk of leukemic transformation differed based on MDS histology. In low-risk MDS histologies, autoimmunity was associated with a 1.9-fold increased risk of leukemia (HR 1.87, 95%CI, 1.17 - 2.99, p=0.008), while no significant effect was seen in other groups. These results suggest that autoimmune disease affects survival in MDS and is associated with decreased mortality. The survival effect was evident in low risk histologies despite higher risk of progression to leukemia. This could represent inflammation-driven hematopoiesis simultaneously favoring less aggressive phenotypes and clonal expansion, which warrants further investigation.Copyright © 2023 American Society of Hematology.