Sjogren Lymphoma Risk — SCE Rheumatology MCQ

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ModerateConnective Tissue DiseaseSjogren Lymphoma RiskSCE Rheumatology

A 55-year-old woman with primary Sjögren syndrome has persistent painless parotid enlargement, anti-Ro antibody positivity and a low serum C4 concentration. Which malignancy is she at greatest increased risk of developing?

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Correct answer: C — Extranodal marginal zone B-cell lymphoma of MALT type

The correct answer is extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) type. Primary Sjögren syndrome confers a substantially increased risk of B-cell non-Hodgkin lymphoma, most characteristically MALT lymphoma arising in a major salivary gland. Persistent parotid enlargement and low C4 are established lymphoma predictors; other warning features include cryoglobulinaemia, palpable purpura, lymphadenopathy, splenomegaly, cytopenias and monoclonal gammopathy. Anti-Ro positivity may contribute to risk models but is less discriminating than persistent glandular swelling and hypocomplementaemia. T-cell lymphoma and chronic lymphocytic leukaemia are not the characteristic Sjögren-associated malignancies. Parotid swelling should not be assumed to represent a salivary epithelial carcinoma, and the assertion that malignancy risk is unchanged is incorrect.

Reference: Clinical picture, outcome and predictive factors of lymphoma in primary Sjögren's syndrome: results from a harmonized dataset (1981–2021), Rheumatology, 2022. https://pubmed.ncbi.nlm.nih.gov/34940812/