Background/Purpose: Sjögren’s disease (SjD) is burdened by significant lymphoproliferative risk. Up to date there is no established consensus on the salivary gland ultrasonographic (SGUS) features of lymphoma or of high lymphoproliferative risk (HLR) lesions (e.g., LELs, GCs, MESA/LESA). The aim is to systematically review existing literature on SGUS features of SjD patients complicated by SG Lymphoma or HLR lesions.

Methods: A systematic review of existing literature (SLR) (January 1988 – January 2025) was conducted according to PRISMA 2020 guidelines (Prospero registration: CRD420251008467).

Results: 15 studies (13 full text articles, 2 international conference abstracts) were included, with a predominantly moderate-low risk of bias (Figure 1). 13/15 described SGUS features of lymphoma, considering 121 total cases. 11/13 described the presence of focal lesions in lymphomas (8/11 quantitatively), identifying 45 focal lesions on 117 cases. These lesions were described with variable features, but commonly presented as a dominant glandular hypo- to anechoic mass on SGUS (Table 1). In 3/11 studies, the parenchyma surrounding the focal lesion was also assessed, with quantitative or inferable SGUS scores indicating moderate to severe involvement in all cases (Table 1). 10/13 studies described a diffuse glandular involvement (presence of SGUS abnormalities) in lymphoma cases (7/10 quantitatively), identifying 32 cases on 105 lymphomas. 4/10 studies quantitatively reported SGUS scores, with moderate-severe involvement in most cases (Table 2). 9/15 studies described HLR lesions, with a total of 161 quantifiable cases. In 4/9 a focal lesion in HLR cases was described, resulting in 11 focal lesions on 16 HLR cases considered, with descriptions and features as heterogeneous as lymphomas. Only 1/4 studies addressed SGUS features of the surrounding parenchyma, reporting a severe grade (Tab.2). 5/9 studies reported a diffuse involvement in the absence of mention of focal lesions in HLR lesions (only 3 quantitatively), identifying 77 cases on 149 HLR lesions. 2/5 of these studies address SGUS scores reporting quantitative measures, more frequently a moderate-severe involvement (Table 2). No prospective studies define SGUS’s role in detecting or predicting lymphoproliferative evolution.

Conclusion: The SLR confirms that both major salivary gland lymphoma and HLR lesions in SjD can appear as a dominant focal lesion (mostly hypo-anechoic lesion/mass), or as a diffuse involvement (mostly moderate-severe SGUS scores). SGUS features are described with highly heterogenous methodology and terminology, thus highlighting the necessity of a standardization in the reporting measures.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/major-salivary-gland-ultrasonographic-features-of-lymphoma-and-high-lymphoproliferative-risk-lesions-in-sjogrens-disease-a-systematic-review/