A 48-year-old woman presents with dry eyes, dry mouth, parotid enlargement, arthralgia, and purpura on the lower limbs. Anti-Ro/SSA and anti-La/SSB antibodies are positive. Her serum protein electrophoresis shows polyclonal hypergammaglobulinemia. Rheumatoid factor is positive at 1:640. Which complication is most concerning in this patient?

• A Pulmonary arterial hypertension
• B Renal tubular acidosis
• C Peripheral neuropathy
• D B-cell lymphoma (MALT or diffuse large B-cell) ✓

Explanation

Primary Sjögren syndrome carries a 15–20-fold increased lifetime risk of non-Hodgkin B-cell lymphoma (predominantly MALT lymphoma of the parotid/salivary glands, or diffuse large B-cell lymphoma). Predictors of lymphoma development include persistent parotid enlargement, cryoglobulinemia, low C4, high-titer rheumatoid factor, and palpable purpura (as seen here). Renal tubular acidosis and peripheral neuropathy are complications but not the most clinically concerning malignant complication.

Reference: Harrison's Principles of Internal Medicine, 21st ed.

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