Purpose
Clinical scenario
Primary Sjogren’s syndrome (pSS) is a progressive, systemic autoimmune disease which most commonly affects women between 30 and 50 years of age, with an estimated prevalence between 0.5% and 1% of the general population [1,2]. It predominantly involves lacrimal and salivary glands (SGs), which are characterized by chronic inflammatory infiltration [3]. This involvement is considered in all classification criteria and usually has an imaging correlate [4].
Moreover, patients affected by pSS have an increased risk of developing an extranodal lymphoma of the major...
Methods and materials
Patients
This study was approved by the referring Institutional review board. Written informed consent was obtained from each patient in accordance with the Declaration of Helsinki and with local guidelines for good clinical practice. In this prospective study, from September 2019 to October 2020, fifteen consecutive patients with definite (ACR-EULAR classification criteria) or clinically suspected pSS and with clinical and/or laboratoristic and/or US findings suspicious for MALT lymphoma were referred to our Institute to undergo a US evaluation of their major SGs followed by US-guided...
Results
The final sample was composed by 9 patients (8 females and 1 male, mean age 67,9 years) with MALT lymphoma diagnosed with US-guided CNB. All SGs were assessed as grade 3 according to OMERACT scoring system.
In 2/9 MALT lymphomas (22,2%) the main feature was the diffuse enlargement of the salivary gland, which had inhomogeneous appearance. 7/9 MALT lymphomas (77,8%) presented themselves as focal areas. Among focal lesions, 7/7 (100%) were very hypoechoic, with an inner appearance that was inhomogeneous in 5/7 (71,4%) and relatively...
Conclusion
In our initial experience, MALT lymphomas developed in major SGs with features of advanced pSS, and usually appeared as focal, hypoechoic, inhomogeneous, hypervascular lesions, with oval shape, well-defined margins and posterior acoustic enhancement. Although the diagnosisofMALT lymphoma is currently based on the histopathological evaluation, our study suggests that there are certain sonographic features that frequently repeat.
Personal information and conflict of interest
F. Tulipano Di Franco:
Nothing to disclose
M. Lorenzon:
Nothing to disclose
A. Zabotti:
Nothing to disclose
S. Zandonella Callagher:
Nothing to disclose
E. Pegolo:
Nothing to disclose
C. Di Loreto:
Nothing to disclose
S. De Vita:
Nothing to disclose
R. Girometti:
Nothing to disclose
C. Zuiani:
Nothing to disclose
References
[1] Maciel G, Crowson CS, Matteson EL, Cornec D. Prevalence of Primary Sjögren’s Syndrome in a US Population-Based Cohort.Arthritis Care & Research. 2017;69(10):1612-1616. doi:10.1002/acr.23173
[2] Mavragani CP, Moutsopoulos HM. Sjögren’s Syndrome.Annual Review of Pathology: Mechanisms of Disease. 2014;9(1):273-285. doi:10.1146/annurev-pathol-012513-104728
[3] Zabotti A, Zandonella Callegher S, Lorenzon M, et al. Ultrasound-guided core needle biopsy compared with open biopsy: a new diagnostic approach to salivary gland enlargement in Sjögren’s syndrome?Rheumatology. Published online September 17, 2020. doi:10.1093/rheumatology/keaa441
[4] Jousse-Joulin S, Milic V, Jonsson MV, et al. Is salivary...