Rheumatoid factor, disease activity may predict lymphoma in primary Sjögren’s syndrome
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The presence of rheumatoid factor and level of disease activity may be risk factors for the development of lymphoma in patients with primary Sjögren’s syndrome, according to recently published research.
Researchers studied 101 patients with primary Sjögren’s syndrome and lymphoma, 86.1% of whom were women with a mean age of 57.4 years at lymphoma diagnosis. Ninety-nine patients had B-cell non-Hodgkin’s lymphoma, and 76 of the patients had marginal zone lymphoma, with 58 cases of mucosa-associated lymphoid tissue type. Lymphomas of the salivary glands were present in 43 patients.
Complete, sustained remission was achieved in 61 patients after initiation of Rituxan (rituximab, Genentech/Biogen Idec) combined with standard doxorubicin, bleomycin, vinblastine and dacarbazine, and 82 achieved remission after additional treatment. Seven patients died, including five from the progression of lymphoma, three of whom presented with diffuse large B-cell lymphoma.
No prior treatments for Sjögren’s syndrome, such as hydroxychloroquine, were associated with the development of lymphoma, but the presence of anti-Sjögren’s syndrome A positivity with or without anti-Sjögren’s syndrome B positivity, presence of rheumatoid factor, cryoglobulinemia, monoclonal component and low C4 levels were linked to development of lymphoma. The risk for lymphoma increased by 5.7% for each additional 10 IU of rheumatoid factor measured, and European League Against Rheumatism (EULAR) Sjögren’s syndrome disease activity index score of 5 or greater was associated with an OR of 3.99. – by Shirley Pulawski
Disclosure: The research was supported by grants from the French Ministry of Health.