Qian Jinyi, Li Lei, Lv Jing, Jiang Yingjie, Ma Qianchen, Pan Haoyu, Wei Xiaohan, Yang Zhixia, Yu Shuyi, Fan Yuying, Teng Jialin, Yang Chengde, Zhang Aifei, Yang Yue, Shi Hui
Department of Rheumatology and Immunology, Shanghai Jiao Tong University Medical School Affiliated Ruijin Hospital, Shanghai, China.
Department of Pathology, Shanghai Jiao Tong University Medical School Affiliated Ruijin Hospital, Shanghai, China.
Lupus Sci Med. 2025 Apr 14;12(1):e001474. doi: 10.1136/lupus-2024-001474.
This study aimed to characterise the clinical features and treatment regimens of patients with lupus who have lymphadenopathy (LAP), as well as to investigate the presence and potential implications of Langerhans cells (LCs) infiltration in lymph nodes.
A case-control study was conducted to identify the clinical characteristics of newly diagnosed, treatment-naïve patients with lupus who have LAP. Lymph node biopsies were performed, and LC infiltration was assessed using immunohistochemical staining for S100, CD1a and Langerin.
A total of 59 patients with SLE who have LAP (SLE-LAP) were enrolled, with 81 patients with SLE without LAP serving as controls. The SLE-LAP group exhibited significantly higher frequencies of fever (64.4% vs 35.8%, p<0.001), anaemia (71.2% vs 42.0%, p<0.001), serous effusion (27.1% vs 11.1%, p=0.015), myositis (10.2% vs 1.2%, p=0.045) and elevated CRP levels (44.1% vs 22.2%, p=0.006). Moreover, autoantibodies, including anti-Smith (37.3% vs 16.0%, p=0.004), anticardiolipin IgG (27.1% vs 11.1%, p=0.015), IgM (42.4% vs 9.9%, p<0.001) and IgA (8.5% vs 0.0%, p=0.027), were more frequently detected in the LAP group. LC infiltration was confirmed in 29 of the 59 lymph node biopsies (49.2%). Immunohistochemical analysis revealed a scattered (58.6%) or focal (41.4%) distribution of LCs. Patients with LC infiltration predominantly presented with fever (72.4%), anaemia (64.3%), skin rashes (62.1%) and arthritis (62.1%). However, no significant differences in clinical manifestations were observed between LC-positive and LC-negative patients.
LC infiltration in the lymph nodes of patients with SLE is relatively common and should be carefully evaluated to prevent misdiagnosis. The role of LCs in the autoimmune response and pathogenesis of SLE warrants further investigation.
本研究旨在描述伴有淋巴结病（LAP）的狼疮患者的临床特征和治疗方案，并调查淋巴结中朗格汉斯细胞（LCs）浸润的存在情况及其潜在影响。
进行了一项病例对照研究，以确定新诊断的、未接受过治疗的伴有LAP的狼疮患者的临床特征。进行了淋巴结活检，并使用针对S100、CD1a和朗格蛋白的免疫组织化学染色评估LC浸润情况。
共纳入59例伴有LAP的系统性红斑狼疮（SLE）患者（SLE-LAP），81例无LAP的SLE患者作为对照。SLE-LAP组发热（64.4%对35.8%，p<0.001）、贫血（71.2%对42.0%，p<0.001）、浆膜腔积液（27.1%对11.1%，p=0.015）、肌炎（10.2%对1.2%，p=0.045）及C反应蛋白水平升高（44.1%对22.2%，p=0.006）的发生率显著更高。此外，LAP组中抗史密斯抗体（37.3%对16.0%，p=0.004）、抗心磷脂IgG（27.1%对11.1%，p=0.015）、IgM（42.4%对9.9%，p<0.001）和IgA（8.5%对0.0%，p=0.027）等自身抗体的检测频率更高。59例淋巴结活检中有29例（49.2%）证实存在LC浸润。免疫组织化学分析显示LC呈散在分布（58.6%）或局灶性分布（41.4%）。LC浸润的患者主要表现为发热（72.4%）、贫血（64.3%）、皮疹（62.1%）和关节炎（62.1%）。然而，LC阳性和LC阴性患者在临床表现上未观察到显著差异。
SLE患者淋巴结中的LC浸润相对常见，应仔细评估以防止误诊。LC在SLE自身免疫反应和发病机制中的作用值得进一步研究。
