Manifestaciones neurológicas del síndrome de Sjögren primario

Clara Méndez Perles, Clara Sangüesa Gómez, Mónica Fernández-Castro, José Luis Andreu Sánchez
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引用次数: 4

Abstract

Primary Sjögren syndrome (PSS) is an autoimmune disease characterized by lymphocytic infiltration in exocrine glands, mainly the salivary and lachrymal glands, resulting in xerophthalmia and xerostomia. Occasionally, PSS has extra-glandular manifestations, including central (CNS) and peripheral nervous system (PNS) involvement. The reported prevalence varies in the literature, mainly because of heterogeneity in the diagnostic and classification criteria. Involvement of the PNS is more frequent than that of the CNS, presenting as axonal, sensory, autonomic or small fiber neuropathies and mononeuritis multiplex. CNS manifestations may be focal or diffuse and are associated with increased extra-glandular involvement and the presence of autoantibodies. Treatment is based on corticosteroids. In severe, refractory- or corticosteroid-dependant cases, immunosuppressant agents such as azathioprine or cyclophosphamide may be useful. Observational studies have suggested that rituximab is useful in some PSS extra-glandular manifestations. Currently, clinical trials with new biological agents such as belimumab and epratuzumab are being conducted in PSS.

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原发性sjogren综合征的神经学表现
原发性Sjögren综合征(Primary Sjögren syndrome, PSS)是一种以淋巴细胞浸润外分泌腺(主要是唾液腺和泪腺)为特征的自身免疫性疾病,导致干眼和口干。偶尔,PSS有腺体外表现,包括中枢(CNS)和周围神经系统(PNS)受累。文献报道的患病率各不相同,主要是因为诊断和分类标准的异质性。累及PNS比CNS更常见,表现为轴突、感觉、自主神经或小纤维神经病变和多发性单神经炎。中枢神经系统的表现可能是局灶性的或弥漫性的,并与腺体外受累增加和自身抗体的存在有关。治疗的基础是皮质类固醇。在严重、难治性或皮质类固醇依赖病例中,免疫抑制剂如硫唑嘌呤或环磷酰胺可能有用。观察性研究表明,利妥昔单抗对一些PSS腺体外表现有用。目前,新的生物制剂如贝利单抗和epratuzumab正在PSS中进行临床试验。
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