Severe relapse after cessation of immunosuppressive therapy in a patient with co-occurrence of neuromyelitis optica spectrum disorder and Sjögren's syndrome: A case report

Ibrain Pub Date : 2024-09-13 DOI:10.1002/ibra.12175
Man-Min Zhu, Zu-Cai Xu, Chang-Yin Yu, Hao Huang
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引用次数: 0

Abstract

Neuromyelitis optica spectrum disorder (NMOSD) is a group of autoimmune disorders characterized by inflammatory involvement of the optic nerve, spinal cord, and central nervous system. NMOSD is often associated with other autoimmune disorders, including Sjogren's syndrome (SS). While NMOSD typically occurs at a peak in young or older individuals, the coexistence of NMOSD and SS in a youngster is rare. Here, we presented a case of a 14-year-old girl with NMOSD and SS who responded well to immunosuppressive therapy but experienced a severe relapse after discontinuation of therapy. We described the clinical course of a case over 8 years, underscoring the importance of long-term treatment for NMOSD and SS. This case, along with the review of relevant literature, will raise awareness of this type of disease and facilitate early diagnosis and treatment to avoid serious sequelae.

Abstract Image

同时伴有视神经脊髓炎谱系障碍和Sjögren综合征的患者停止免疫抑制治疗后严重复发:1例报告
视神经脊髓炎谱系障碍(NMOSD)是一组自身免疫性疾病,其特征是炎症累及视神经、脊髓和中枢神经系统。NMOSD通常与其他自身免疫性疾病相关,包括干燥综合征(SS)。虽然NMOSD通常发生在年轻人或老年人中,但NMOSD和SS在年轻人中共存是罕见的。在这里,我们报告了一个14岁的NMOSD和SS女孩,她对免疫抑制治疗反应良好,但在停止治疗后经历了严重的复发。我们描述了一个病例超过8年的临床过程,强调了长期治疗NMOSD和SS的重要性。该病例以及相关文献的回顾将提高人们对这类疾病的认识,促进早期诊断和治疗,避免严重的后遗症。
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