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Sjögren disease lymphoma risk — SCE Rheumatology MCQ

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HardConnective tissue diseasesSjögren disease lymphoma riskSCE Rheumatology

A 63-year-old woman with primary Sjögren disease develops unilateral parotid enlargement, night sweats and palpable purpura. C4 is low, rheumatoid factor is positive and serum cryoglobulins are detected. Schirmer testing had previously confirmed severe sicca symptoms. What is the most important complication to screen for?

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Correct answer: CB-cell lymphoma

Persistent salivary gland enlargement, purpura, low C4 and cryoglobulinaemia in Sjögren disease are red flags for B-cell lymphoma. Giant cell arteritis may cause systemic symptoms but would not explain this Sjögren-specific risk pattern. The teaching point is that lymphoma risk stratification is central in follow-up of Sjögren disease.

Reference: BSR Sjögren disease guideline 2023