New advances in the classification, pathogenesis and treatment of Sjogren's syndrome

Abstract

In the current review, we summarize the newly proposed classification criteria for Sjogren's syndrome, recent findings of Sjogren's syndrome pathogenesis and the latest achievements in disease management. A new set of Sjogren's syndrome classification criteria has been recently proposed by an expert consensus panel of the American College of Rheumatology-Sjögren's International Collaborative Clinical Alliance. Recent findings reveal new aspects in the activation of innate and adaptive immune pathways and novel animal models - highly reminiscent of human Sjogren's syndrome-are described. Of particular note, apoptosis of epithelial cells as a result of deficient IκBζ, previously shown to be a modulator of NFκB activity, has been suggested as a central pathogenetic event. Mechanistic data on anti-B-cell therapies, gene transfer approaches aimed to restore secretory function, as well mesenchymal stem cell transplantation in mice and humans are also discussed. Over the last year, a new set of classification criteria for Sjogren's syndrome has been suggested, new Sjogren's syndrome-like animal models have been described and significant progress has been made in understanding the activation of innate and adaptive immune responses. New therapeutic approaches have been also implemented with variable success.