Sjogren Syndrome — SCE Rheumatology MCQ

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ModerateConnective Tissue DiseaseSjogren SyndromeSCE Rheumatology

A 50-year-old woman with long-standing primary Sjögren disease presents with progressively enlarging, painless unilateral parotid swelling, ipsilateral facial nerve palsy and an elevated serum beta-2 microglobulin concentration. Which diagnosis is the priority to exclude?

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Correct answer: B — Salivary-gland MALT lymphoma

The priority diagnosis is salivary-gland B-cell lymphoma, particularly extranodal marginal-zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma). Sjögren disease confers a markedly increased risk of non-Hodgkin lymphoma, and persistent unilateral parotid enlargement is a major warning feature; facial nerve dysfunction raises concern for an infiltrative parotid lesion. Elevated beta-2 microglobulin provides additional, although nonspecific, evidence of lymphoproliferative or systemic activity. Sialolithiasis usually causes meal-related pain and intermittent swelling. IgG4-related disease more often produces painless bilateral salivary enlargement, commonly involving the submandibular glands. Sarcoidosis can cause parotid swelling and facial palsy in Heerfordt syndrome, but usually has supporting features such as uveitis, fever or thoracic disease. Tuberculosis requires compatible exposure and granulomatous or constitutional features.

Reference: Price EJ et al. Executive summary: British Society for Rheumatology guideline on management of adult and juvenile onset Sjögren disease. Rheumatology. 2024/2025. https://pubmed.ncbi.nlm.nih.gov/38785300/