Jaskólska Marta, Rytlewska Magdalena, Dułak Natalia Aleksandra, Ulanowski Miłosz, Kwarciany Mariusz, Wiglusz Mariusz Stanisław, Cubała Wiesław Jerzy, Chmielewski Michał
Department of Rheumatology, Clinical Immunology, Geriatrics and Internal Medicine, Medical University of Gdansk, Gdansk, Poland.
Department of Adult Neurology, Medical University of Gdansk, Gdansk, Poland.
Rheumatol Int. 2025 Jan 21;45(2):35. doi: 10.1007/s00296-024-05753-8.
Sjogren's disease (SjD) is a chronic and disabling autoimmune disease, predominantly characterized by dryness of the mouth and eyes, resulting from lymphocytic infiltration of exocrine glands. While these are the most prominent symptoms, extra-glandular manifestations are also common. Studies suggest that up to 70% of SjD patients experience neurological symptoms, which interestingly often precede the hallmark dryness. Although every structure of the nervous system can be affected, disorders of peripheral nervous system (PNS) are more common than central nervous system (CNS) involvement. The CNS manifestations can range from subtle to severe, with some patients experiencing a rapid progression of symptoms. The literature documents cases where patients initially diagnosed with Creutzfeldt-Jakob disease, neurosarcoidosis, temporary hemiplegia, neuromyelitis optica, or epilepsy were ultimately diagnosed as having SjD. Here, we present five SjD patient cases, each with a different manifestation of CNS involvement, along with an overview of the current understanding of CNS disorders in the course of the disease. In four cases, the neurological manifestations appeared before the onset of sicca symptoms. In one patient, diagnosis was delayed by 15 years due to an atypical presentation. After receiving immunosuppression, all patients experienced notable alleviation of their symptoms.
干燥综合征（SjD）是一种慢性致残性自身免疫性疾病，主要特征为口腔和眼部干燥，由外分泌腺淋巴细胞浸润所致。虽然这些是最突出的症状，但腺体外表现也很常见。研究表明，高达70%的干燥综合征患者会出现神经症状，有趣的是，这些症状往往先于标志性的干燥症状出现。虽然神经系统的每个结构都可能受到影响，但周围神经系统（PNS）疾病比中枢神经系统（CNS）受累更为常见。中枢神经系统表现可轻可重，有些患者症状进展迅速。文献记载了一些病例，最初被诊断为克雅氏病、神经结节病、短暂性偏瘫、视神经脊髓炎或癫痫的患者最终被诊断为干燥综合征。在此，我们展示5例干燥综合征患者病例，每例都有不同的中枢神经系统受累表现，并概述目前对该疾病过程中中枢神经系统疾病的认识。在4例病例中，神经症状出现在干燥症状发作之前。在1例患者中，由于表现不典型，诊断延迟了15年。接受免疫抑制治疗后，所有患者的症状均有明显缓解。
