Abstract
Primary Sjögren’s syndrome (pSS) is a chronic and heterogeneous disorder characterized by a wide spectrum of glandular and extra-glandular features. The hallmark of pSS is considered to be the immune-mediated involvement of the exocrine glands and B-cell hyperactivation. This leads pSS patients to an increased risk of developing lymphoproliferative diseases, and persistent (>2 months) major salivary gland enlargement is a well-known clinical sign of possible involvement by B cell lymphoma. Better stratification of the patients may improve understanding of the mechanism underlying the risk of lymphoproliferative disorder. Here, we summarize the role of different imaging techniques and a bioptic approach in pSS patients, focusing mainly on the role of salivary gland ultrasonography (SGUS) and a US-guided core needle biopsy (Us-guided CNB) as diagnostic and prognostic tools in pSS patients with persistent parotid swelling.
Highlights
Lymphoma Risk in pSS Patients, and the Role of Persistent Salivary Gland Swelling Primary Sjögren’s syndrome is a chronic systemic autoimmune disease, affecting mainly middle-aged perimenopausal women with a female to male ratio of 9:1, and has an estimated prevalence of about 0.5% in the general population [1]
The most represented non-Hodgkin’s lymphoma (NHL) histotype in pSS is an indolent, low-grade extra-nodal marginal zone (MZ) B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) that is mainly localized in major salivary glands (SGs), and the parotid in particular, or, less frequently, in lacrimal glands, lungs and stomach; some could transform into high-grade lymphoma, mainly diffuse large B-cell lymphoma, which may originate from MZ NHL [5,6,7,8,9]
The results demonstrated that US-guided CNB, inclusive of flow cytometry, is a useful diagnostic tool, providing sufficient material to discern a range of SG pathologic findings in pSS, and allowing a definitive pathological diagnosis and a clinical decision making without further testing
Summary
Lymphoma Risk in pSS Patients, and the Role of Persistent Salivary Gland Swelling Primary Sjögren’s syndrome (pSS) is a chronic systemic autoimmune disease, affecting mainly middle-aged perimenopausal women with a female to male ratio of 9:1, and has an estimated prevalence of about 0.5% in the general population [1]. Persistent SGs swelling, especially parotid enlargement, is considered a major risk factor for the development of MALT lymphoma [12], as highlighted by Kassan SS et al in 1978 [1]. The Authors focused on ultrasonography (US) as one of the most relevant imaging techniques for the examination of parotid swelling and of its most dreadful consequence, i.e., the development of SGs lymphoma, in pSS, followed by Magnetic Resonance Imaging (MRI). The SGUS scores are set and tested for diagnostic purposes, and only few data are published on their roles for follow-up, prediction of treatment response or as surrogate of glandular damage detected by histopathology [24] Regarding this last item, SGUS scores and damage-related lesions have been reported as being correlated with objective SGs function (i.e., reduced salivary flow rate) [36]. Published initial studies report very encouraging data to this end [24,48,49]
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