Zehrfeld Nadine, Witte Torsten, Ernst Diana
Klinik für Immunologie und Rheumatologie, Medizinische Hochschule Hannover, Carl-Neuberg-Str. 1, 30625, Hannover, Deutschland.
Z Rheumatol. 2024 Apr;83(3):217-228. doi: 10.1007/s00393-024-01493-z. Epub 2024 Mar 18.
Sjögren's syndrome (SjS) is the most common connective tissue disease with a prevalence of 1:200. Predominantly affecting women, SjS is associated with destruction of the exocrine glands, leading to xerophthalmia and xerostomia. In over 50% of patients, there are also extraglandular manifestations, leading to multiple organ manifestations including polyneuropathies and interstitial lung disease as well as symptoms such as fatigue and arthralgia. Diagnostic procedures include biomarkers, in particular anti-SS-A/Ro antibodies, histology of salivary glands, and salivary gland sonography. There are currently no licensed immunosuppressive drugs for SjS, so current treatment is often based on off-label use of drugs. The European League Against Rheumatism (EULAR) has recently published treatment recommendations based on the prevailing organ manifestations. Several promising controlled trials with novel compounds and concepts are currently in progress.
干燥综合征(SjS)是最常见的结缔组织病,患病率为1:200。SjS主要影响女性,与外分泌腺破坏有关,导致干眼症和口干症。超过50%的患者还存在腺体外表现,导致多器官表现,包括多发性神经病和间质性肺病以及疲劳和关节痛等症状。诊断方法包括生物标志物,特别是抗SS-A/Ro抗体、唾液腺组织学检查和唾液腺超声检查。目前尚无用于SjS的获批免疫抑制药物,因此目前的治疗通常基于药物的非标签使用。欧洲抗风湿病联盟(EULAR)最近根据主要的器官表现发布了治疗建议。目前正在进行几项关于新型化合物和概念的有前景的对照试验。