Keywords:
Haematologic, Head and neck, Salivary glands, Ultrasound, Ultrasound-Colour Doppler, Education, Haematologic diseases, Lymphoma
Authors:
F. Tulipano Di Franco, M. Lorenzon, A. Zabotti, S. Zandonella Callagher, E. Pegolo, C. Di Loreto, S. De Vita, R. Girometti, C. Zuiani; Udine/IT
DOI:
10.26044/ecr2021/C-10893
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 SGs, most often of the mucosa-associated lymphoid tissue type (MALT) [5]. As a reference, it has been recently reported that the B-cell lymphomas occur in 5%-10% of pSS patients, and the risk increases by 2,2% per year of age [6], with a 7-19-fold increased relative risk of developing non-Hodgkin’s lymphoma (NHL) compared to the general population [7].
Role of ultrasounds
Among imaging techniques (i.e. sialography, MRI, US etc.), Ultrasound (US) has been proven to be a non-invasive and accessible tool with high sensitivity for echostructural abnormalities of major SGs [4], able to improve the diagnostic performance of American_European Consensus Group (AECG) and American College of Rheumatology (ACR) criteria [8].
In fact, US not only provides useful, real-time information of the involvement of SGs at diagnosis, but also plays a crucial role in monitoring disease activity [4]. This is of particular importance, given the progressive nature of the disease and considering the enhanced risk for patients with pSS of developing MALT, as mentioned above. However, to the best of our knowledge, sonographic features of MALT lymphoma of major SGs have not been assessed in literature.
Purpose
We aim to describe the sonographic features of a series of MALT lymphomas of the major SGs in patients with Sjogren syndrome, diagnosed with US-guided core-needle biopsy (CNB).