Background The clinical coexistence of Systemic Lupus Erythematosus (SLE) and Sjögrens Syndrome (SS) was recognized in 1959. The prevalence of SS among patients with SLE varies considerably among the published studies (10%–30%). There is still controversy as to whether or not SLE patients with overlapping SS have a distinct and significantly milder lupus. To address the clinical and serologic features of SLE and differences from SLE that occurs in overlap with SS.
Methods This is a multicenter, descriptive, cross-sectional study of 3575 patients from the Spanish Society for Rheumatology Lupus Registry (RELESSER). Unselected SLE patients from 45 Rheumatology Departments across Spain were evaluated for the presence of overlapping SS using the American-European consensus criteria. Cumulative clinical data were collected at the moment of the last assessment. Clinical and laboratory parameters in SLE patients with SS (SLEwSS) were compared with those in SLE patients without SS (SLEwoSS).
Results SS was identified in 516 SLE patients (14.4%). Compared with the SLEwoSS group, patients with SLEwSS were significantly older, had a higher frequency of mucocutaneous manifestations, Raynauds phenomenon, peripheral neuropathy, anti-Ro/SSA, anti-La/SSB, neoplasia, and older age at death, but had a significantly lower frequency of renal involvement, thrombocytopenia, anti-dsDNA, anti-2-GPI IgM and complement consumption. Both groups displayed a clinically similar presentation of lymphadenopathy, systemic vasculitis, serositis, damage accrual, mortality, musculoskeletal and CNS manifestations.
Conclusions SLEwSS appears to constitute a subgroup of SLE patients with distinct clinical and serologic features, in whom SS is expressed as an overlapping entity. A particular cluster of clinical variables, namely, mucocutaneous manifestations, Raynauds phenomenon, peripheral neuropathy, renal involvement and thrombocytopenia, was found to be important overall for discriminating SLE patients with or without SS. SLEwSS patients constitute a subgroup of patients with SLE characterized by milder lupus: older age at death, similar rates of mortality and SLICC-ACR damage index, less renal and immunological manifestations.
Funding Source(s): None
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