摘要目的:探讨抗黑色素瘤分化相关基因5（MDA5）抗体阳性皮肌炎（MDA5-DM）合并侵袭性肺曲霉菌病（IPA）的临床特征、危险因素及预后。方法:收集2020年1月至2022年6月于中日友好医院风湿免疫科住院治疗的MDA5-DM患者55例，根据是否合并IPA分为2组：IPA（+）组（14例）和IPA（-）组（41例）。回顾性分析患者微生物学检查结果及临床资料。2组正态分布计量资料比较采用 t检验，非正态分布的计量资料比较采用Mann-Whitney U检验，计数资料比较采用 χ2检验。危险因素分析采用二元Logistic回归，生存分析采用Kaplan-Meier法。 结果:IPA（+）组患者以感染黄曲霉（5/14，35.7%）和烟曲霉（4/14，28.6%）最为常见。与IPA（-）组相比，IPA（+）组患者具有更高的α羟丁酸脱氢酶[246.0（160.3，328.3）U/L与191.0（167.0，227.5）U/L， Z=-2.02， P=0.043]和血清铁蛋白[1 306.7（518.7，2 977.8）ng/ml与472.6（269.0，792.1）ng/ml， Z=-2.09， P=0.036]，更低的CD8 +T细胞计数[111.5（68.3，214.0）×10 6/L与188.0（141.0，270.0）×10 6/L， Z=-2.18， P=0.029），且支气管肺泡灌洗液半乳糖甘露聚糖试验阳性更多见[70.0%（7/10）与18.9%（7/37）， χ2=9.82， P=0.004]。多因素分析发现血清铁蛋白升高是发生IPA的独立危险因素[调整后 OR值（95% CI）=1.001（1.000，1.002）， P=0.031]。生存分析结果示IPA（+）组的6个月累积生存率显著低于IPA（-）组（78.6%与97.6%， P=0.021）。 结论:MDA5-DM患者感染IPA后死亡率高，血清铁蛋白升高是发生IPA的独立危险因素，积极预防和治疗IPA有望改善患者预后。

abstractsObjective:To investigate the clinical characteristics, risk factors, and prognosis of invasive pulmonary aspergillosis (IPA) in patients with dermatomyositis associated with positive anti-melanoma differentiation-associated gene 5 (MDA5-DM).Methods:A total of 55 patients with MDA5-DM were analyzed. Patients were divided into IPA (+) group (14 cases) and IPA (-) group (41 cases) based on the presence of IPA. Microbiological examination and clinical data were analyzed. Risk factor analysis was performed using Binary Logistic regression, and survival analysis was carried out using Kaplan-Meier method.Results:Aspergillus flavus (5/14, 35.7%) and Aspergillus fumigatus (4/14, 28.6%) were the most common species in MDA5-DM patients with IPA. Compared to the IPA (-) group, IPA (+) group had higher serum level of α-hydroxybutyrate dehydrogenase (246 U/L vs. 191 U/L, Z=-2.02, P=0.043) and ferritin [1 306.7(518.7, 2 977.8)ng/ml vs. 472.6(269.0, 792.1)ng/ml, Z=-2.09, P=0.036], lower CD8 + T lymphocyte counts {[111.5 (68.3, 214.0)]×10 6/L vs. [188.0(141.0, 270.0)]×10 6/L, Z=-2.18, P=0.029}, and more positive BALF GM tests [70.0%(7/10) vs. 18.9%(7/37), χ2=9.82, P=0.004]. Elevated serum ferritin was found to be an independent risk factor for IPA occurrence [adjusted OR (95% CI)=1.001 (1.000, 1.002), P=0.031)]. In addition, the 6-month cumulative survival rate was significantly lower in the IPA (+) group than in the IPA (-) group (78.6% vs. 97.6%, P=0.021). Conclusion:The mortality of MDA5-DM patients is increased after IPA infection. Elevated serum ferritin is an independent risk factor for IPA occurrence, and active prevention and treatment of IPA are expected to improve the prognosis of patients.