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Sjogren Syndrome — SCE Rheumatology MCQ

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ModerateConnective Tissue DiseaseSjogren SyndromeSCE Rheumatology

A 48-year-old woman with primary Sjögren disease asks which findings identify a group at increased risk of future lymphoma. According to the British Society for Rheumatology guideline, which combination consists entirely of predictors that consistently emerge for future lymphoma development?

Educational content. Not a substitute for clinical judgement or local policy.

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Correct answer: A — Persistent salivary gland enlargement, low C4, cryoglobulinaemia and monoclonal gammopathy

Explanation lettering: D = shown as B · E = shown as C · B = shown as D · C = shown as E

A is correct. The BSR guideline identifies salivary gland enlargement, hypocomplementaemia (particularly low C4), cryoglobulinaemia and monoclonal gammopathy among factors consistently associated with subsequent lymphoma in Sjögren disease. Lymphadenopathy and a high salivary-gland focus score are further predictors. These findings should prompt a lower threshold for assessment for lymphoproliferative disease, particularly where gland enlargement is persistent or new. Anti-Ro/La antibodies, fatigue, sicca symptoms, arthralgia and raised ESR are common disease features or nonspecific markers and are not included in the guideline’s consistently emerging predictor set. Polyclonal hypergammaglobulinaemia indicates B-cell activation but is not equivalent to monoclonal gammopathy.

Reference: British Society for Rheumatology. Guideline for the management of adult and juvenile onset Sjögren disease (predictors of lymphoma), 2023 guideline published 2024. https://www.rheumatology.org.uk/guidelines/clinicalguidelines/adultsratnfguideline/view