Verstappen Gwenny M, Kroese Frans G M, Bootsma Hendrika
Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands.
Rheumatology (Oxford). 2021 Jul 1;60(7):3088-3098. doi: 10.1093/rheumatology/kez004.
A histologic hallmark of primary SS (pSS) is lymphocytic infiltration of the salivary and lacrimal glands, in particular by CD4+ T and B cells. In the early stages of the disease, infiltrates are dominated by CD4+ T cells, while B cell accumulation occurs at later stages. Activated T cells contribute to pathogenesis by producing pro-inflammatory cytokines and by inducing B cell activation, which results in the establishment of a positive feedback loop. In the inflamed glandular tissues, many different CD4+ effector subsets are present, including IFN-γ-producing Th1 cells, IL-17-producing Th17 cells and IL-21-producing T follicular helper cells. In blood from pSS patients, frequently observed abnormalities of the T cell compartment are CD4+ T cell lymphopenia and enrichment of circulating follicular helper T (Tfh) cells. Tfh cells are critical mediators of T cell-dependent B cell hyperactivity and these cells can be targeted by immunotherapy. Inhibition of T cell activation, preferably early in the disease process, can mitigate B cell activity and may be a promising treatment approach in this disease.
原发性干燥综合征（pSS）的组织学特征是唾液腺和泪腺的淋巴细胞浸润，尤其是CD4 + T细胞和B细胞浸润。在疾病早期，浸润以CD4 + T细胞为主，而B细胞在后期积聚。活化的T细胞通过产生促炎细胞因子和诱导B细胞活化来促进发病机制，这导致建立正反馈回路。在发炎的腺组织中，存在许多不同的CD4 +效应子亚群，包括产生IFN-γ的Th1细胞、产生IL-17的Th17细胞和产生IL-21的T滤泡辅助细胞。在pSS患者的血液中，经常观察到的T细胞区室异常是CD4 + T细胞淋巴细胞减少和循环滤泡辅助性T（Tfh）细胞增多。Tfh细胞是T细胞依赖性B细胞过度活跃的关键介质，这些细胞可成为免疫治疗的靶点。抑制T细胞活化，最好在疾病过程的早期，可减轻B细胞活性，可能是这种疾病一种有前景的治疗方法。
