Chen Xueqin, Wang Jia, Chen Caifeng, Wang Jundan, Du Lei, Qin Wenxin, Xu Ziyu, Liu Lei, Qin Xiude, Kong Fanxin
The Fourth Clinical Medical College, Guangzhou University of Chinese Medicine, Shenzhen, People's Republic of China.
Department of Traditional Chinese Medicine, The Fifth Medical Center, Chinese PLA General Hospital, Beijing, People's Republic of China.
Infect Drug Resist. 2025 Aug 14;18:4109-4118. doi: 10.2147/IDR.S534677. eCollection 2025.
Opportunistic infections pose significant challenges in patients undergoing immunosuppressive treatment for autoimmune disorders. Neuromyelitis optica spectrum disorder (NMOSD) is a rare disabling autoimmune condition requiring both acute attack management and long-term relapse prevention. B-cell depleting therapies, such as inebilizumab have demonstrated efficacy in reducing relapse rates and disability progression in NMOSD. Here, we present the first known NMOSD case who developed Pneumocystis jirovecii pneumonia following maintenance therapy with inebilizumab, showing a probable association (Naranjo score=6). We explored the underlying mechanisms linking B-cell depletion to Pneumocystis jirovecii, including antibody-dependent and immunomodulatory pathways. Furthermore, we provided an overview of Pneumocystis jirovecii pneumonia in autoimmune central nervous system disorders and summarized its clinical features, previous medication use, and prognostic factors. We advocate for risk-adapted prophylaxis to reduce morbidity and mortality associated with opportunistic infections in NMOSD.
机会性感染给接受自身免疫性疾病免疫抑制治疗的患者带来了重大挑战。视神经脊髓炎谱系障碍（NMOSD）是一种罕见的致残性自身免疫性疾病，需要对急性发作进行管理并长期预防复发。B细胞耗竭疗法，如inebilizumab，已证明在降低NMOSD的复发率和残疾进展方面有效。在此，我们报告首例已知在接受inebilizumab维持治疗后发生耶氏肺孢子菌肺炎的NMOSD病例，显示出可能的关联（Naranjo评分=6）。我们探讨了将B细胞耗竭与耶氏肺孢子菌联系起来的潜在机制，包括抗体依赖性和免疫调节途径。此外，我们概述了自身免疫性中枢神经系统疾病中的耶氏肺孢子菌肺炎，并总结了其临床特征、既往用药情况和预后因素。我们提倡采用风险适应性预防措施，以降低NMOSD中与机会性感染相关的发病率和死亡率。
