• Alessia Alunno, Maria Comasia Leone, Elena Bartoloni, Roberto Gerli, and Francesco Carubbi.
• Rheumatology Unit, University of Perugia, Perugia, Italy.
• Panminerva Med. 2020 Dec 4.

IntroductionPrimary Sjögren's syndrome (pSS) is a systemic autoimmune disease characterized by a chronic inflammatory process mainly affecting the exocrine glands but also burdened by a wide range of extraglandular manifestations. Non-Hodgkin lymphoma (NHL) is the most severe pSS complication worsening disease prognosis.Evidence AcquisitionWe summarized original articles published between April 2018 and May 2020 on this topic aiming to highlight novelties on lymphoma and lymphomagenesis.Evidence SynthesisResults have been grouped by epidemiology, etiopathogenesis and predictors of lymphoma.ConclusionsNHL is the most severe complication of pSS and occurs in around 5-10% of patients. Over the last two years, several clinical, serological, and histopathological features have been proposed as predictive for lymphoma in pSS patients, allowing early diagnosis and consequently, better management and prognosis. Individual monitoring for disease activity and possible lymphoma development is a central clue in the evaluation of pSS patients.

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