Medicine · Rheumatology (SLE, RA, Vasculitis, Crystal Arthropathies, Scleroderma)

A 55-year-old woman with a 10-year history of RA on methotrexate and etanercept develops dry eyes, dry mouth, parotid enlargement, and positive anti-Ro and anti-La antibodies. Which additional autoantibody, if strongly positive, would raise concern for lymphoma development?

  • A Anti-CCP (cyclic citrullinated peptide)
  • B Anti-centromere antibody
  • C Anti-dsDNA antibody
  • D Cryoglobulinemia (type II, rheumatoid factor + monoclonal IgM)
Correct answer: D. Cryoglobulinemia (type II, rheumatoid factor + monoclonal IgM)

Explanation

In primary Sjögren's syndrome (this presentation indicates overlapping secondary Sjögren's), the development of type II cryoglobulinemia (mixed cryoglobulinemia with monoclonal IgM rheumatoid factor plus polyclonal IgG) is a strong risk marker for progression to MALT lymphoma (marginal zone B-cell lymphoma), the most common lymphoma in Sjögren's. Other risk factors for lymphoma in Sjögren's include persistent parotid enlargement, palpable purpura, low C4, and high Foci score on biopsy. Anti-CCP is specific for RA, anti-centromere for limited scleroderma, and anti-dsDNA for SLE.

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

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

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