Athanasios Tzioufas and colleagues recently published a study in The Lancet Rheumatology

Sjogren’s disease (SjD) is a systemic autoimmune disorder with a notable predisposition to lymphoma development, particularly mucosa-associated lymphoid tissue (MALT) lymphoma.

Various risk factors for lymphoma development in SjD have been proposed; however, existing studies are significantly limited for two primary reasons: (i) they tend to treat all lymphomas related to SjD as a single entity, and (ii) they lack clearly defined temporal markers distinguishing the onset of SjD from the initiation of lymphoma. Consequently, some features observed in SjD patients could be attributed either to an underlying lymphoma that is progressing slowly or to SjD itself, leading to confusion regarding whether these features are true predictors of lymphoma or simply manifestations of an already present lymphoma.

In this study, we showed that rheumatoid factor positivity at the time of Sjögren's disease diagnosis serves as the most reliable and temporally distant independent predictor of MALT lymphoma development. Furthermore, rheumatoid factor-positive patients with Sjögren's disease who also had high systemic disease activity (European League Against Rheumatism Sjögren's syndrome disease activity index≥5) showed an increased risk for MALT lymphoma. Additionally, we found that the proposed MALT lymphoma predictors of cryoglobulinemia, salivary gland enlargement, hypocomplementemia, and palpable purpura actually represent a more advanced stage of Sjögren's disease-related lymphomagenesis, rather than typical lymphoma risk factors at Sjögren's disease diagnosis. Thus, the occurrence of such manifestations should signify a warning for more intense follow-up and diagnostic testing to exclude an already existing underlying lymphoma.

Our findings provide a clinically applicable roadmap on how to manage patients with Sjögren's disease, who are prone to develop the most commonly encountered histological type of MALT lymphoma. Patients with Sjögren's disease should be evaluated at the time of diagnosis for the presence of rheumatoid factor, and ideally a minor salivary gland biopsy should be done to rule out underlying MALT lymphoproliferation.

Pubmed

Identification and evolution of predictors of Sjögren's disease-associated mucosa-associated lymphoid tissue lymphoma development over time: a case-control study
Goules AV, Chatzis L, Pezoulas VC, Patsouras M, Mavragani C, Quartuccio L, Baldini C, De Vita S, Fotiadis DI, Tzioufas AG. Lancet Rheumatol. 2024 Aug 22:S2665-9913(24)00183-8. doi: 10.1016/S2665-9913(24)00183-8. Online ahead of print.PMID: 39182505