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Abstract：
OBJECTIVE: The aim of this study was to assess the histopathological features of the parotid glands in patients with paediatric-onset Sjögren\'s disease (pedSjD) in comparison to patients with adult-onset Sjögren\'s disease (adSjD).
METHODS: This study was performed in Groningen, the Netherlands. Patients with pedSjD from a diagnostic paediatric cohort (n=19), patients with adSjD from a diagnostic adult cohort (n=32) and patients with adSjD who participated in a clinical trial (n=42) with a baseline parotid gland biopsy were included. Parotid gland biopsies were analysed after (immuno)histological staining for SjD-related histopathological markers and compared between groups.
RESULTS: All characteristic histopathological features of adSjD were also observed in pedSjD. There were no significant differences in lymphoepithelial lesions or immunoglobulin A (IgA)/IgG plasma cell shift between the pedSjD and the adSjD cohorts. However, compared with the diagnostic adSjD cohort (with comparable total EULAR Sjögren\'s Syndrome Disease Activity Index (ESSDAI) scores), pedSjD showed more severe lymphocytic infiltration as reflected by a higher focus score (p=0.003), a higher relative surface area of CD45+ infiltrate (p=0.041), higher numbers of B and T lymphocytes/mm2 (p=0.004 and p=0.029, respectively), a higher B/T lymphocyte ratio (p=0.013), higher numbers of CD21+ follicular dendritic cell networks/mm2 (p=0.029) and germinal centres (GC)/mm2 (p=0.002). Compared with the trial adSjD cohort, with significant higher total ESSDAI scores (p=0.001), only the B/T lymphocyte ratio and numbers of GC/mm2 were significantly higher in the pedSjD cohort (p=0.023 and p=0.018, respectively).
CONCLUSIONS: Patients with pedSjD exhibit more pronounced histopathological features compared with patients with adSjD at diagnosis. Notably, the histopathology of patients with pedSjD aligns more closely with that observed in an adSjD clinical trial cohort, with even stronger B lymphocyte involvement.
METHODS: This study was performed in Groningen, the Netherlands. Patients with pedSjD from a diagnostic paediatric cohort (n=19), patients with adSjD from a diagnostic adult cohort (n=32) and patients with adSjD who participated in a clinical trial (n=42) with a baseline parotid gland biopsy were included. Parotid gland biopsies were analysed after (immuno)histological staining for SjD-related histopathological markers and compared between groups.
RESULTS: All characteristic histopathological features of adSjD were also observed in pedSjD. There were no significant differences in lymphoepithelial lesions or immunoglobulin A (IgA)/IgG plasma cell shift between the pedSjD and the adSjD cohorts. However, compared with the diagnostic adSjD cohort (with comparable total EULAR Sjögren\'s Syndrome Disease Activity Index (ESSDAI) scores), pedSjD showed more severe lymphocytic infiltration as reflected by a higher focus score (p=0.003), a higher relative surface area of CD45+ infiltrate (p=0.041), higher numbers of B and T lymphocytes/mm2 (p=0.004 and p=0.029, respectively), a higher B/T lymphocyte ratio (p=0.013), higher numbers of CD21+ follicular dendritic cell networks/mm2 (p=0.029) and germinal centres (GC)/mm2 (p=0.002). Compared with the trial adSjD cohort, with significant higher total ESSDAI scores (p=0.001), only the B/T lymphocyte ratio and numbers of GC/mm2 were significantly higher in the pedSjD cohort (p=0.023 and p=0.018, respectively).
CONCLUSIONS: Patients with pedSjD exhibit more pronounced histopathological features compared with patients with adSjD at diagnosis. Notably, the histopathology of patients with pedSjD aligns more closely with that observed in an adSjD clinical trial cohort, with even stronger B lymphocyte involvement.
摘要：
目的：本研究的目的是评估儿科发病的Sjögren病（pedSjD）患者与成年发病的Sjögren病（adSjD）患者腮腺的组织病理学特征。
方法：这项研究在格罗宁根进行，荷兰。来自儿科诊断队列的pedSjD患者（n=19），纳入了诊断性成人队列中的adSjD患者(n=32)和参与一项有基线腮腺活检的临床试验的adSjD患者(n=42).在对SjD相关的组织病理学标志物进行（免疫）组织学染色后分析腮腺活检，并在组间进行比较。
结果：在pedSjD中也观察到了adSjD的所有特征性组织病理学特征。pedSjD和adSjD队列之间的淋巴上皮病变或免疫球蛋白A（IgA）/IgG浆细胞移位没有显着差异。然而,与诊断性adSjD队列（具有可比的EULARSjögren综合征疾病活动指数（ESSDAI）总评分）相比，pedSjD显示更严重的淋巴细胞浸润，反映在更高的焦点评分（p=0.003），较高的CD45+渗透物的相对表面积（p=0.041），B和T淋巴细胞数量/mm2较高（分别为p=0.004和p=0.029），B/T淋巴细胞比率较高（p=0.013），更多的CD21+滤泡树突状细胞网络/mm2(p=0.029)和生发中心(GC)/mm2(p=0.002).与试验adSjD队列相比，ESSDAI总分明显较高（p=0.001），在pedSjD队列中，只有B/T淋巴细胞比率和GC/mm2数量显着升高（分别为p=0.023和p=0.018）。
结论：与adSjD患者相比，pedSjD患者在诊断时表现出更明显的组织病理学特征。值得注意的是,pedSjD患者的组织病理学与adSjD临床试验队列中观察到的组织病理学更接近，更强烈的B淋巴细胞参与。
方法：这项研究在格罗宁根进行，荷兰。来自儿科诊断队列的pedSjD患者（n=19），纳入了诊断性成人队列中的adSjD患者(n=32)和参与一项有基线腮腺活检的临床试验的adSjD患者(n=42).在对SjD相关的组织病理学标志物进行（免疫）组织学染色后分析腮腺活检，并在组间进行比较。
结果：在pedSjD中也观察到了adSjD的所有特征性组织病理学特征。pedSjD和adSjD队列之间的淋巴上皮病变或免疫球蛋白A（IgA）/IgG浆细胞移位没有显着差异。然而,与诊断性adSjD队列（具有可比的EULARSjögren综合征疾病活动指数（ESSDAI）总评分）相比，pedSjD显示更严重的淋巴细胞浸润，反映在更高的焦点评分（p=0.003），较高的CD45+渗透物的相对表面积（p=0.041），B和T淋巴细胞数量/mm2较高（分别为p=0.004和p=0.029），B/T淋巴细胞比率较高（p=0.013），更多的CD21+滤泡树突状细胞网络/mm2(p=0.029)和生发中心(GC)/mm2(p=0.002).与试验adSjD队列相比，ESSDAI总分明显较高（p=0.001），在pedSjD队列中，只有B/T淋巴细胞比率和GC/mm2数量显着升高（分别为p=0.023和p=0.018）。
结论：与adSjD患者相比，pedSjD患者在诊断时表现出更明显的组织病理学特征。值得注意的是,pedSjD患者的组织病理学与adSjD临床试验队列中观察到的组织病理学更接近，更强烈的B淋巴细胞参与。
