Rheumatology Clinic, Scripps Memorial Hospital and Research Foundation, La Jolla, CA.
Strasbourg University Hospital, National Center for Rare Systemic Autoimmune Diseases, Strasbourg, France.
Rheumatology (Oxford). 2021 May 14;60(5):2066-2074. doi: 10.1093/rheumatology/kez142.
SS is usually described as having severe fatigue, dryness, diffuse pain, glandular swelling, and various extraglandular (systemic) manifestations. Clinical trials have generally failed because the vast majority of enrolled patients had no extraglandular manifestations at the time of enrolment but suffered from fatigue, dryness and pain that did not significantly respond to the study medication. A number of hypotheses on the pathogenesis of pSS have been put forward, including disturbances of innate and adaptive immunity as well as abnormalities of the interface between immune disorders and the neuro-endocrine system related to lacrimal and secretory gland dysfunction. Thus, future therapies must be designed for improvement of the symptoms of dry eyes and dry mouth, extraglandular disease, and fatigue and cognitive deficits. Given the inadequacies and limitations of current treatment options, we suggest that innovative directions involving interactions with neuroscientists and neuropsychiatrists together or combined with new immune targeting may be hold promise for better treating pSS.
SS 通常被描述为严重疲劳、干燥、弥漫性疼痛、腺体肿胀和各种腺体外（全身）表现。临床试验通常失败，因为绝大多数入组患者在入组时没有腺体外表现，但患有疲劳、干燥和疼痛，对研究药物没有明显反应。已经提出了关于 pSS 发病机制的许多假设，包括先天和适应性免疫紊乱以及与泪腺和分泌腺功能障碍相关的免疫紊乱与神经内分泌系统之间界面的异常。因此，未来的治疗方法必须设计用于改善干眼和口干、腺体外疾病以及疲劳和认知缺陷的症状。鉴于当前治疗选择的不足和限制，我们建议涉及与神经科学家和神经精神科医生相互作用或与新的免疫靶向相结合的创新方向可能有希望更好地治疗 pSS。
