Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Centre Groningen, The Netherlands.
Department of Rheumatology and Clinical Immunology, and Department of Pathology and Medical Biology, University of Groningen, University Medical Centre Groningen, The Netherlands.
Clin Exp Rheumatol. 2018 May-Jun;36 Suppl 112(3):222-233. Epub 2018 Aug 14.
The formation of lymphomononuclear cell infiltrates organising as periductal infiltrates in the salivary glands of patients with primary Sjögren's syndrome (pSS) is one of the hallmarks of the disease. Historically, the clinical role of salivary gland histopathology, most commonly performed on labial salivary gland biopsies, has been confined to the clinical classification and diagnosis of pSS whereby according to the ACR-EULAR a positive histopathology finding is a requirement for the diagnosis of pSS in the absence of anti-Ro/SSA antibodies. In recent years, further understanding of the heterogeneity of the immune cell infiltration and organisation within the salivary glands of pSS patients and its correlation with clinical manifestations of the disease has led to propose salivary gland histopathology as a novel tool able to identify patients at higher risk of developing more severe extraglandular manifestations and lymphoma. Furthermore, recent clinical developments in ongoing randomised clinical trials with novel biologics in pSS have focused on salivary glands histopathology to inform on patients stratification based on target validation, proof of drug efficacy and mechanisms of response/resistance to therapy. However, lack of standardisation of methodology and analysis has hindered the reproducibility of data from different groups and no definitive evidence in support of the use of salivary glands histopathology to inform clinical management of patients with pSS has been provided. In this review, we summarise recent evidence highlighting the promises and pitfalls of salivary glands histopathology in pSS emphasising the need for an international consensus on standardisation of methodology with validation in large prospective multicentre initiatives.
原发性干燥综合征（pSS）患者的唾液腺中形成以导管周围浸润为特征的淋巴单核细胞浸润是该疾病的标志之一。从历史上看，唾液腺组织病理学的临床作用主要局限于唇腺活检，用于 pSS 的临床分类和诊断，即根据 ACR-EULAR 标准，如果没有抗 Ro/SSA 抗体，则阳性组织病理学发现是 pSS 诊断的必要条件。近年来，对 pSS 患者唾液腺中免疫细胞浸润和组织的异质性及其与疾病临床表现的相关性的进一步了解，促使人们提出唾液腺组织病理学作为一种新的工具，能够识别出发生更严重的腺外表现和淋巴瘤风险更高的患者。此外，pSS 中新型生物制剂的正在进行的随机临床试验中的最近临床进展也集中在唾液腺组织病理学上，以根据目标验证、药物疗效证据以及对治疗的反应/耐药机制对患者进行分层。然而，由于缺乏方法学和分析的标准化，不同组别的数据的可重复性受到阻碍，也没有明确的证据支持使用唾液腺组织病理学来告知 pSS 患者的临床管理。在这篇综述中，我们总结了最近的证据，强调了唾液腺组织病理学在 pSS 中的前景和陷阱，强调需要在大型前瞻性多中心研究中达成关于方法学标准化和验证的国际共识。
