Primary Sjögren syndrome carries a recognised risk of B-cell lymphoma. When low-grade lymphoma develops — including MALT lymphoma, marginal zone lymphoma, small lymphocytic lymphoma, or lymphoplasmacytic lymphoma — management must be tailored to the specific histological subtype and disease stage, and diverges from standard Sjögren syndrome care.
This protocol addresses patients with Sjögren syndrome who have developed low-grade B-cell lymphoma: MALT lymphoma, marginal zone lymphoma (MZL), small lymphocytic lymphoma (SLL), or lymphoplasmacytic lymphoma (LPL). These subtypes account for the large majority of lymphoma cases reported in primary Sjögren syndrome. The clinical approach is individualised according to lymphoma subtype, disease stage, and the presence or absence of systemic involvement or constitutional symptoms.
For eligible patients, close monitoring without immediate intervention is among the strategies considered — particularly when lymphoma involvement is limited and systemic features are absent. A treatment-based approach may be appropriate for others depending on disease extent.
DOI: 10.1136/annrheumdis-2019-216114
Three subtypes of B-cell lymphoma account for more than 90% of reported cases in primary SjS: mucosa-associated lymphoid tissue (MALT) lymphoma, other marginal zone lymphomas (MZL) and Diffuse large B cell lymphoma.
Treatment of B-cell lymphoma should be individualised according to the specific histological subtype and disease stage.
For primary SjS-2002 patients diagnosed with low grade haematological neoplasia, some clinicians recommend a watchful waiting approach when lymphoma only affects the exocrine glands, especially in the absence of constitutional symptoms, systemic features or B-cell activation biomarkers.
For patients with marginal zone lymphomas, small lymphocytic lymphoma (SLL) and lymphoplasmacytic lymphoma (LPL) in early disease stages (in particular, stage I or non-bulky stage II), treatment may include radiotherapy (with or without chemotherapy), although a watch-and-wait strategy could be an alternative to spare the side effects of therapy.
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