Abstract
Sjögren's syndrome (SS) is a chronic, systemic, inflammatory autoimmune disease characterized by lymphocyte proliferation and progressive damage to exocrine glands. The diagnosis of SS is challenging due to its complicated clinical manifestations and non-specific signs. Salivary gland biopsy plays an important role in the diagnosis of SS, especially with anti-Sjögren's syndrome antigen A (SSA) and anti-SSB antibody negativity. Histopathology based on biopsy has clinical significance for disease stratification and prognosis evaluation, such as risk assessment for the development of non-Hodgkin's lymphoma. Furthermore, histopathological changes of salivary gland may be implicated in evaluating the efficacy of biological agents in SS. In this review, we summarize the histopathological features of salivary gland, the mechanism of histopathological changes and their clinical significance, as well as non-invasive imaging techniques of salivary glands as a potential alternative to salivary gland biopsy in SS.
Highlights
Sjögren’s syndrome (SS) is a chronic, systemic, inflammatory autoimmune disease characterized by lymphocyte proliferation and progressive damage to exocrine glands
Haacke et al reported that germinal center (GC) formation is not a risk factor for non-Hodgkin’s lymphoma (NHL) but a reflection of high disease activity, because their controlled trial showed no significant difference in GCs between SS patients with and without parotid mucosaassociated lymphoid tissue (MALT) [50]
This study revealed that the absolute pre-treatment number of CD20+ B cells/mm2 in the parotid gland parenchyma are predictive for the responsiveness to rituximab therapy in patients with SS as defined by ESSDAI, suggesting a potential role of parotid gland biopsy (PGB) in guiding personalized treatment
Summary
Sjögren’s syndrome (SS) is a chronic, systemic, inflammatory autoimmune disease characterized by lymphocyte proliferation and progressive damage to exocrine glands. In addition to impairment of salivary gland and lacrimal gland function, SS is frequently accompanied by multiple serum autoantibodies and systemic organ involvement that has a huge impact on the long-term quality of life of patients [1]. Salivary gland histopathology based on salivary gland biopsy plays an important role in the diagnosis of SS and broadly applied in clinical practice [4]. Minor salivary gland biopsy is the most common and the best method to determine the salivary gland composition in SS in clinical setting due to its disease specificity, wide availability, and minimal invasiveness. According to the classification criteria for SS, labial salivary gland biopsy (LSGB) is mandatory, especially with anti-SSA and anti-SSB antibody negativity [5,6,7]. In this review, we focus on the histopathological features and the mechanism of histopathological changes of LSGB, as well as their clinical significance in SS
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