Konen Franz Felix, Seeliger Tabea, Schwenkenbecher Philipp, Gingele Stefan, Jendretzky Konstantin Fritz, Sühs Kurt-Wolfram, Ernst Diana, Witte Torsten, Skripuletz Thomas
Department of Neurology, Hannover Medical School, Carl-Neuberg-Straße 1, 30625 Hannover, Germany.
Department of Immunology & Rheumatology, Hannover Medical School, Carl-Neuberg-Straße 1, 30625 Hannover, Germany.
Biomedicines. 2022 Oct 3;10(10):2470. doi: 10.3390/biomedicines10102470.
Sjögren's syndrome (SS) is an autoimmune disease characterized by sicca symptoms and various extra-glandular manifestations. The diagnosis of SS requires sicca symptoms, anti-SSA(Ro)-antibody positivity, and/or pathological focus scores on a minor salivary gland biopsy. Previous studies have investigated different biomarkers in order to avoid invasive diagnostic procedures. It was found that kappa and lambda free light chains (KFLC and LFLC) in saliva are specific for SS. FLC concentrations in saliva and serum were determined in 130 patients-50 with SS and neurological involvement (Neuro-Sjögren) and 80 neurological controls. The EULAR SS disease activity index and patient reported index (ESSPRI) were determined in patients with SS. Patients with SS revealed increased pain and decreased saliva production according to the ESSPRI and Saxon test, respectively, with increasing FLC concentrations in the saliva. No significant differences in serum and salivary protein concentrations were observed between patients with SS and controls. KFLC and LFLC concentrations in saliva are not suitable to distinguish patients with Neuro-Sjögren and neurological control subjects, thus a diagnostic biopsy is still required. The association of salivary KFLC and LFLC concentrations with saliva production and ESSPRI pain score suggests a complex relationship between dryness and pain in patients with SS.
干燥综合征（SS）是一种自身免疫性疾病，其特征为干燥症状和各种腺体外表现。SS的诊断需要干燥症状、抗SSA（Ro）抗体阳性，和/或小唾液腺活检的病理灶性评分。既往研究已对不同生物标志物进行了调查，以避免侵入性诊断程序。研究发现，唾液中的κ和λ游离轻链（KFLC和LFLC）对SS具有特异性。对130例患者（50例患有SS且有神经受累（神经型干燥综合征）和80例神经科对照者）的唾液和血清中的FLC浓度进行了测定。对SS患者测定了欧洲抗风湿病联盟（EULAR）SS疾病活动指数和患者报告指数（ESSPRI）。根据ESSPRI和撒克逊试验，SS患者分别显示疼痛加剧和唾液分泌减少，且唾液中FLC浓度增加。SS患者与对照组之间在血清和唾液蛋白浓度方面未观察到显著差异。唾液中的KFLC和LFLC浓度不适用于区分神经型干燥综合征患者和神经科对照者，因此仍需要进行诊断性活检。唾液KFLC和LFLC浓度与唾液分泌及ESSPRI疼痛评分之间的关联提示SS患者的干燥与疼痛之间存在复杂关系。
