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Sjögren-Associated Lymphoma — RACP Adult Medicine MCQ

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HardRheumatologySjögren-Associated LymphomaRACP Adult Medicine

A 50-year-old woman with primary Sjögren syndrome presents with bilateral parotid swelling, lymphadenopathy, and a rising serum IgM level with a monoclonal band. She has a history of recurrent parotitis. What complication should be most strongly suspected?

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Correct answer: C — Marginal zone lymphoma (MALT lymphoma)

Patients with primary Sjögren syndrome have a 15–20 times increased risk of developing non-Hodgkin lymphoma, particularly extranodal marginal zone B-cell lymphoma (MALT lymphoma) of the parotid glands. Warning signs include persistent parotid swelling, lymphadenopathy, palpable purpura, low C4, cryoglobulinaemia, and rising monoclonal immunoglobulin. Biopsy is essential for diagnosis.

Reference: eTG – 2025 – Rheumatology; EULAR – 2020 – Sjögren Syndrome Recommendations