Abstract:In recent years, a significant progress has been made in the research on neuromyelitis optica spectrum disorders (NMOSD) and its associations with connective tissue diseases (CTD) such as Sj?gren’s syndrome (SS) and systemic lupus erythematosus (SLE). Particularly, a deeper understanding of the immunological mechanisms, genetic backgrounds, and environmental factors related to NMOSD, along with insights into the pathological connections between SS, SLE, and NMOSD, has provided new perspectives for accurate diagnosis and effective treatment. This is especially evident in the identification of biomarkers and improvements in imaging techniques.The coexistence of NMOSD and CTD poses complex challenges for clinical diagnosis and treatment. Therefore, there is a need for more refined and comprehensive diagnostic methods and treatment strategies, with interdisciplinary collaboration playing a crucial role in enhancing diagnostic accuracy and treatment effectiveness. Future research should focus on developing more precise diagnostic technologies and tailored treatment plans to improve the prognosis and quality of life for this particular patient population.