PLEURISY IN PRIMARY SJOGRENS-SYNDROME - T-CELL RECEPTOR BETA-CHAIN VARIABLE REGION GENE BIAS AND LOCAL AUTOANTIBODY PRODUCTION IN THE PLEURAL EFFUSION

Pleurisy with or without effusion has not been considered to be associ ated with primary Sjogren's syndrome (SS), but rather to represent a m anifestation of the underlying disorder, usually rheumatoid arthritis in secondary SS. We describe a patient with primary SS who presented w ith pleural effusions (PE) as an initial manifestation. Serological st udies of paired serum and PE specimens demonstrated the occurrence of local immune reactions in the pleura, including the production of rheu matoid factor and anti-SS-A antibody, the formation of immune complexe s, and activation of complement. In addition, the analysis of T cell r eceptor beta-chain variable (V beta) regions in the PE revealed the ov erexpression of a number of V beta gene products, including V beta 2 a nd V beta 13 that have previously been shown to be overrepresented in the salivary glands of patients with SS. Thus, our report not only cal ls for an awareness of pleurisy as an extraglandular manifestation of primary SS, but suggests that a common biased T cell response might pl ay a critical role in the pathogenesis of the glandular as well as ext raglandular manifestations.