REVIEW   

Panminerva Medica 2021 December;63(4):491-8

DOI: 10.23736/S0031-0808.20.04079-3

Copyright © 2020 EDIZIONI MINERVA MEDICA

language: English

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

Alessia ALUNNO ¹, Maria C. LEONE ², Elena BARTOLONI ¹, Roberto GERLI ¹, Francesco CARUBBI ³ ✉

¹ Unit of Rheumatology, University of Perugia, Perugia, Italy; ² Unit of Rheumatology, S. Maria Hospital, Terni, Italy; ³ Unit of COVID-19 Medicine, Department of Medicine, ASL1 Avezzano-Sulmona-L’Aquila, L’Aquila, Italy

Primary 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. We summarized original articles published between April 2018 and May 2020 on this topic aiming to highlight novelties on lymphoma and lymphomagenesis. Results have been grouped by epidemiology, etiopathogenesis and predictors of lymphoma. NHL 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.

KEY WORDS: Sjögren’s Syndrome; Lymphoma, non-Hodgkin; Lymphoma, B-cell, marginal zone; B-lymphocytes