原发性干燥综合征(primary Sjögren’s syndrome, pSS)的中枢神经系统受累少见, 常表现为脑白质病变、视神经脊髓炎谱系疾病(neuromyelitis optica spectrum disorder, NMOSD)或横贯性脊髓炎。NMOSD是一类免疫介导的中枢神经系统炎性脱髓鞘病变, 发病特点为高复发、高致残。pSS合并NMOSD患者病情更严重、预后更差。本研究报道1例pSS合并NMOSD妊娠期危重症的病例, 患者为有pSS病史的30岁孕妇, 被诊断为NMOSD, 在妊娠期接受甲泼尼龙琥珀酸钠、丙种球蛋白和硫酸羟氯喹片联合治疗, 治疗后患者出现口干、吞咽困难、腰背以下部位麻木感基本消失。患者于院外未规律服药, 右下肢出现无力、不能活动, 左下肢活动尚可, 偶伴麻木感。后急诊入院, 紧急行剖宫产分娩一健康男婴后, 患者病情加重, 高热伴双下肢瘫痪无力, 无法自主大小便。患者再次接受甲泼尼龙琥珀酸钠、丙种球蛋白治疗, 但症状改善不佳。首次给予利妥昔单抗输注后患者出现尿路感染, 予控制感染后继续规律输注利妥昔单抗, 后期患者行走略跛, 能够控制排尿和排便, 可正常工作。该病例表明, pSS合并NMOSD的妊娠期患者可考虑给予甲泼尼龙琥珀酸钠、丙种球蛋白和硫酸羟氯喹片联合治疗。利妥昔单抗可以明显改善NMSOD患者产后瘫痪、二便失禁等症状, 但同时可能有感染的风险。

Central nervous system involvement in primary Sjögren's syndrome (pSS) is less common and usually presents as white matter lesions, neuromyelitis optica spectrum disorder (NMOSD), or transverse myelitis. NMOSD is an immune-mediated inflammatory demyelinating disease of the central nervous system with a high rate of relapse and significant disability. Studies have shown that patients with pSS combined with NMOSD have more severe symptoms and poorer prognosis. Here, we present a case of critical illness in pregnancy-associated NMOSD combined with Sjögren's syndrome. The patient was a 30-year-old pregnant woman with a history of Sjögren's syndrome who was diagnosed with NMOSD. She received combination therapy with steroids, intravenous immunoglobulin (IVIG), and hydroxychloroquine during pregnancy, resulting in partial resolution of numbness below the waist. However, due to irregular medication adherence outside the hospital setting, she developed weakness in her right lower limb accompanied by inability to move it, while her left lower limb still had some mobility but occasional numbness along with urinary and fecal incontinence. Ten days later, she was admitted to the emergency department where an emergency cesarean section was performed to deliver a healthy baby boy. However, her condition worsened postpartum as she developed high fever accompanied by bilateral lower limb paralysis and weakness along with loss of voluntary control over urination and defecation. The patient underwent ano-ther course of treatment consisting of steroids and IVIG; however there was limited improvement in symptoms observed after this intervention. Following administration of rituximab for the first time, the patient developed urinary tract infection which was successfully managed before continuing regular infusions. In later stages the patient could walk slightly with a limp and regained control over urination and defecation, allowing her to resume normal activities. This case suggests that combination therapy with steroids, IVIG, and hydroxychloroquine should be considered for the patients with pregnancy-associated NMOSD combined with Sjögren's syndrome. Rituximab can significantly improve symptoms such as postpartum paralysis in patients with NMOSD, however, there may be a risk of infection associated with its use.