视神经脊髓炎谱系疾病合并干燥综合征1例文献复习

Review of a Case of Optic Neuromyelitis Spectrum Disease Complicated with Sjogren’s Syndrome

视神经脊髓炎谱系疾病(neuromyelitis optica spectrum disorders, NMOSD)是一组主要由体液免疫参与的抗原–抗体介导的CNS炎性脱髓鞘疾病谱,常青壮年起病,以女性多见,致残率高预后差。原发性干燥综合症(primary Sjogren’s Syndrome, pSS)是累及多种外分泌腺体的慢性炎症性自身免疫病,临床上常侵犯涎腺和泪腺,表现为口、眼干燥症,可有包括神经系统在内的多器官、多系统的损害。目前关于NMOSD合并pSS的报道较少,研究证明NMOSD合并pSS患者病情更严重,预后更差,且容易误诊、漏诊。本文通过分析一例NMOSD合并pSS患者的诊治过程,并查阅文献,报道如下,以提高广大临床医生对此类疾病的认识,能够及早诊断,规范治疗,改善预后。

Neuromyelitis optica spectrum disorders (NMOSD) is a group of antigen-antibody-mediated CNS inflammatory demyelinating diseases that are mainly involved in humoral immunity. The disease begins in ever young adults and is more common in women. The disability rate is high and the prognosis is poor. Primary Sjogren’s Syndrome (pSS) is a chronic inflammatory autoimmune disease involving a variety of exocrine glands. It often invades salivary glands and lacrimal glands in clinic, manifesting as dry mouth and eyes, and may include the nervous system damage to multiple organs and multiple systems within. At present, there are few reports about NMOSD combined with pSS. Studies have shown that patients with NMOSD combined with pSS have more serious disease, worse prognosis, and are easy to be misdiagnosed and missed. This article analyzes the diagnosis and treatment process of a patient with NMOSD and pSS, and consults the literature. The report is as follows, in order to improve clinicians’ understanding of this type of disease, and enable early diagnosis, standardized treatment, and improved prognosis.