Objective: To describe the clinical characteristics and to explore the prognostic factors of concurrent pneumocystis jirovecii pneumonia (PJP) in patients with idiopathic inflammatory myopathy (IIM). Methods: We retrospectively enrolled consecutive IIM patients diagnosed with PJP at our center between January 2014 and December 2022. Fifty-eight IIM-PJP patients were enrolled in our study, with the age of 26-79 (56.3±12.1) years. There were 19 males (32.9%) and 39 females (67.1%). The participants were classified into survival group (21 patients) and non-survival group (37 patients) according to the clinical outcome. Cox regression models were used to analyze the prognostic factors. Results: Compared with the survival group, there were more patients with anti- melanoma differentiation-associated gene (MDA5) positive, respiratory failure, hospital-acquired pneumonia, second-line PJP treatment, and caspofungin treatment in non-survival group (P<0.05). Meanwhile, the time from onset of IIM, corticosteroid therapy, and immunosuppressant medication to diagnosis of PJP was significantly shorter in non-survival patients than survival patients (P<0.05). Additionally, non-survival patients had higher neutrophil percentage and creatine kinase (P<0.05), and lower lymphocyte count and percentage, platelets, minimal albumin, NK cell count, T cell count, and CD8+T cell count (P<0.05). Multivariate analysis showed that anti-MDA5 positive (HR=3.54, P<0.05, 95%CI: 1.27-9.89), oral candidiasis infection (HR=2.62, P<0.05, 95%CI: 1.07-6.43) and thrombocytopenia (HR=5.00, P<0.01, 95%CI: 1.88-13.32) were independent risk factors for IIM-PJP patients. Conclusions: Middle-aged females were the main IIM-PJP in our group, and the mortality rate was 63.8%. Anti-MDA5 antibody positive, oral candidiasis infection, and thrombocytopenia were independent survival risk factors for them.
目的： 分析特发性炎性肌病（IIM）合并肺孢子菌肺炎（PJP）患者的临床特征并探究其预后相关因素。 方法： 回顾性分析2014年1月至2022年12月在北京协和医院住院接受诊疗的IIM合并PJP患者临床资料。共纳入IIM合并PJP患者58例，其中男性19例（32.9%）、女性39例（67.1%），年龄26~79（56.3±12.1）岁。根据临床结局将研究对象分为存活组（21例）及死亡组（37例），通过Cox回归模型分析IIM合并PJP患者的预后相关因素。 结果： 死亡组抗黑色素瘤分化相关基因-5（MDA5）抗体阳性、呼吸衰竭、合并院内获得性肺炎、采用二线PJP治疗、卡泊芬净治疗的患者多于存活组（均P<0.05）；死亡组患者的原发病起病至感染起病时间、糖皮质激素治疗至感染起病时间、免疫抑制剂治疗至感染起病时间均显著短于存活组（均P<0.05）；死亡组患者外周血中性粒细胞比例及肌酸激酶高于存活组（均P<0.05），淋巴细胞计数及比例、血小板、最低白蛋白、NK细胞计数、T细胞计数、CD8+T细胞计数低于存活组（均P<0.05）。多因素分析后发现，仅抗MDA5抗体阳性（HR=3.54，P<0.05，95%CI：1.27~9.89），合并口腔念珠菌感染（HR=2.62，P<0.05，95%CI：1.07~6.43）和血小板降低（HR=5.00，P<0.01，95%CI：1.88~13.32）是IIM合并PJP患者生存的独立危险因素。 结论： 本组IIM患者合并PJP以中年女性为主，病死率高达63.8%。抗MDA5抗体阳性、口腔念珠菌感染、血小板降低可能是IIM合并PJP患者预后的独立危险因素。.