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Parotid Lymphoma Risk Sjogren — SCE Rheumatology MCQ

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HardConnective Tissue DiseaseParotid Lymphoma Risk SjogrenSCE Rheumatology

A 45-year-old woman with rheumatoid arthritis and associated Sjögren disease develops painless bilateral parotid enlargement that persists for 10 weeks, with a firm, more prominent left parotid gland. She is afebrile and has no tenderness, dental symptoms or meal-related pain. Investigations show an ESR of 85 mm/hour, polyclonal hypergammaglobulinaemia and repeatedly low serum C4. Which diagnosis is most important to exclude?

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Correct answer: BMALT-type extranodal marginal zone B-cell lymphoma

The diagnosis to exclude is a MALT-type extranodal marginal zone B-cell lymphoma of the parotid. Persistent major salivary-gland enlargement and low C4 are established lymphoma predictors in Sjögren disease; a new firm or asymmetric component is particularly concerning. Polyclonal hypergammaglobulinaemia reflects B-cell activation but is less specific as an independent predictor. Benign lymphoepithelial sialadenitis can cause gland enlargement, but persistence and asymmetry with hypocomplementaemia require investigation for malignant transformation. IgG4-related disease may cause painless salivary enlargement but is less compatible with established Sjögren disease and low C4. Viral parotitis is usually acute, while obstructive sialadenitis typically causes episodic meal-related pain. Salivary-gland imaging followed by appropriately targeted tissue diagnosis should be arranged if lymphoma is suspected.

Reference: Price EJ et al. British Society for Rheumatology guideline on management of adult and juvenile onset Sjögren disease. Rheumatology. 2025;64:409–439. https://pubmed.ncbi.nlm.nih.gov/24899571/