Background Primary Sjögren’s syndrome (pSS) is the autoimmune disease most often associated with lymphoma. Mixed cryoglobulinemia as well as persistent parotid swelling are the well known pre-lymphomatous clinical conditions.
Objectives To describe the prevalence of lymphoproliferative complications (defined as B-cell lymphoma or definite conditions predisposing to lymphoma, i.e, cryoglobulinemic vasculitis (CV) and major salivary gland swelling) in a cohort of pSS patients followed in five Rheumatologic Centres.
Methods Demographic, clinical, laboratory and histopathologic data in 1170 pSS were retrospectively collected according to a standard protocol. Univariate analyses were performed.
Results Prevalence of lymphoma in this SS cohort was 4.4% (51/1170), prevalence of CV (1) was 3.9% (33/850), and prevalence of salivary gland swelling and/or myoepithelial syaladenitis was 30.9% (362/1170). Salivary gland swelling and/or MESA, CV and lymphoma shared many laboratory features, i.e., positive rheumatoid factor (RF), hypocomplementemia and leucopenia, as well as a the presence of purpura as clinical hallmark of the circulating immune complexes (table 1). Interestingly, polyclonal hypergammaglobulinemia was strictly associated with salivary gland swelling, but it was not associated with CV or lymphoma; on the other hand, serum monoclonal component was significantly associated with CV or lymphoma, but not with salivary gland swelling and/or MESA.
Conclusions Salivary gland swelling and CV are confirmed as red flags for lymphoma evolution in pSS and are strictly linked (2). Polyclonal hypergammaglobulinemia is not a red flag for lymphoma in pSS. B-cell clonal proliferation from polyclonal to monoclonal likely occurs in the target tissue of pSS (e.g. salivary gland MALT tissue), predisposing to CV and/or to lymphoma. Cryoglobulinemia marks the emergence of abnormal RF-positive B-cell clones, but MALT lymphoma may also occur in the absence of cryoglobulinemia.
De Vita S et al. Ann Rheum Dis 2011
De Vita S, et al. Rheumatology 2012
Disclosure of Interest None Declared
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