Novel insights on lymphoma and lymphomagenesis in primary Sjögren's Syndrome

Alunno, Alessia; Leone, Maria Comasia; Bartoloni, Elena; Gerli, Roberto; Carubbi, Francesco

doi:10.23736/s0031-0808.20.04079-3

Cited by 10 publications

(7 citation statements)

References 40 publications

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“…Persistent major salivary gland (SG) enlargement is a major clinical risk factor for B-cell lymphoma, and it may signify an established lymphoma stage, a prelymphomatous stage or even a completely different disease 6 7. The parotid glands are the main site of B-cell lymphoma development among patients with pSS, and biopsies of the major SGs are reserved for patients with pSS suspected of having glandular lymphoma, mainly based on SG swelling.…”

Section: Introductionmentioning

confidence: 99%

Safety, patient acceptance and diagnostic accuracy of ultrasound core needle biopsy of parotid or submandibular glands in primary Sjögren’s syndrome with suspected salivary gland lymphoma

et al. 2022

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BackgroundEnlargement of the major salivary glands (SGs) is a major risk factor for B-cell lymphoma among patients with primary Sjögren’s syndrome (pSS). Ultrasound-guided core needle biopsy (US-guided CNB) could be a novel technique to manage SG enlargement among patients with pSS.ObjectiveAccordingly, this study’s main aim was to evaluate the safety, patient tolerance and diagnostic accuracy of US-guided CNB procedure for patients with pSS with major SG enlargement.MethodsPatients with clinical diagnosis of pSS and a clinical indication for SG biopsy consecutively underwent US-guided CNB between September 2019 and June 2021. These patients were evaluated clinically 1, 2 and 12 weeks after US-guided CNB. Patients were asked to complete a questionnaire about postprocedural complications as well as periprocedural pain, using the Visual Analogue Scale. Complications were categorised as transient (<12 weeks) or persistent (≥12 weeks).ResultsUS-guided CNB was performed on 30 major salivary glands (22 parotid glands and 8 submandibular glands). The procedure was well tolerated. Transient complications—such as haematoma, swelling—were observed among 43% of patients, and mean periprocedural pain was low. However, no persistent complications were reported during the study’s follow-up period.ConclusionUS-guided CNB represents a novel approach for the management of patients with pSS with SG enlargement. The procedure showed remarkable patient safety and tolerance, allowing adequate glandular sampling and a definite diagnosis for almost all participating patients without long-term complications.

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Section: Introductionmentioning

confidence: 99%

Safety, patient acceptance and diagnostic accuracy of ultrasound core needle biopsy of parotid or submandibular glands in primary Sjögren’s syndrome with suspected salivary gland lymphoma

et al. 2022

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“… 172 , 175 – 177 Their usefulness has been confirmed through the years, with variable strength of association dependent on study population characteristics and methods of assessment. 178 – 180 The correlation with ESSDAI has also been an object of debate, as the presence of specific clinical and biological manifestations (i.e. persistent major SG enlargement and cryoglobulinaemia) has proven to be useful in identifying pSS patients at a higher risk of lymphoma evolution, rather than the overall disease activity evaluated by ESSDAI.…”

Section: Lymphoma In Pssmentioning

confidence: 99%

“…Some novel biomarkers of pSS-related lymphoproliferation have been studied in recent years ( Table 2 ), but the lack of external validation and prospective evaluation, as well as the intrinsic complexity of lymphoma pathogenesis, still limits their clinical use as single predictive factors. 14 , 179 , 180 , 184 …”

Section: Lymphoma In Pssmentioning

confidence: 99%

Sjögren syndrome: looking forward to the future

Callegher

Giovannini

Zenz

et al. 2022

Therapeutic Advances in Musculoskeletal

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Primary Sjögren’s syndrome (pSS) is a heterogeneous disease characterised by a wide spectrum of manifestations that vary according to the different stages of the disease and among different subsets of patients. The aim of this qualitative literature review is to summarise the recent advances that have been reported in pSS, ranging from the early phases to the established disease and its complications. We analysed the diagnostic, prognostic, and management aspects of pSS, with a look into future clinical and research developments. The early phases of pSS, usually antedating diagnosis, allow us to investigate the pathophysiology and risk factors of the overt disease, thus allowing better and timely patient stratification. Salivary gland ultrasound (SGUS) is emerging as a valid complementary, or even alternative, tool for histopathology in the diagnosis of pSS, due to a standardised scoring system with good agreement and performance. Other promising innovations include the application of artificial intelligence to SGUS, ultrasound-guided core needle biopsy, and a wide array of novel diagnostic and prognostic biomarkers. Stratifying pSS patients through the integration of clinical, laboratory, imaging, and histopathological data; differentiating between activity-related and damage-related manifestations; and identifying patients at higher risk of lymphoma development are essential steps for an optimal management and individualised treatment approach. As new treatment options are emerging for both glandular and systemic manifestations, there is a need for a more reliable treatment response evaluation. pSS is a complex and heterogeneous disease, and many distinct aspects should be considered in the different stages of the disease and subsets of patients. In recent years, efforts have been made to improve our understanding of the disease, and certainly in the coming years, some of these novelties will become part of our routine clinical practice, thus improving the management of pSS patients.

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“…Non-Hodgkin's B-cell Lymphoma (NHL) is one of the major complications of pSS, representing the leading cause of patient decreased survival (80)(81)(82). It occurs in 5-7% of primary and secondary SS patients, who carry a 7-fold increased risk of developing lymphoma than the normal population (83).…”

Section: Lymphoma and Other Malignanciesmentioning

confidence: 99%

Chest high-resolution computed tomography in primary Sjögren's syndrome: an up-to-date primer for rheumatologists

Cereser

Giovannini

Caronia

et al. 2022

Clinical and Experimental Rheumatology

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Pulmonary manifestations, including airway involvement and interstitial lung disease, are the most frequent extra-glandular complications of primary Sjögren's syndrome (pSS). Chest high-resolution computed tomography (HRCT) is a cornerstone of pulmonary diagnostic imaging, aiming to detect, characterise, and quantify such conditions. In patients with pSS-related lung abnormalities, HRCT proved helpful in various clinical scenarios, including baseline and follow-up evaluation, assessment of superimposed infections, suspected progressive interstitial lung diseases, and acute exacerbation. This review aims to provide a primer for rheumatologists on chest HRCT, illustrating the up-to-date technique, imaging findings, and clinical indications in pSS and highlighting the importance of rheumatologist-radiologist constructive collaboration in the clinical management of such patients. Chest HRCT examination State-of-art techniqueRadiologists should perform chest HRCT according to updated recommendations to make the most of its potential, as highlighted in Table I (8,(19)(20)(21)(22)(23). Anatomo-radiologic correlationChest HRCT interpretation should rely on a basic understanding of pulmonary anatomy (19). In this regard, the sec-

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Novel insights on lymphoma and lymphomagenesis in primary Sjögren's Syndrome

Cited by 10 publications

References 40 publications

Safety, patient acceptance and diagnostic accuracy of ultrasound core needle biopsy of parotid or submandibular glands in primary Sjögren’s syndrome with suspected salivary gland lymphoma

Safety, patient acceptance and diagnostic accuracy of ultrasound core needle biopsy of parotid or submandibular glands in primary Sjögren’s syndrome with suspected salivary gland lymphoma

Sjögren syndrome: looking forward to the future

Chest high-resolution computed tomography in primary Sjögren's syndrome: an up-to-date primer for rheumatologists

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