Traitement des atteintes systémiques de la maladie de Sjögren

Primary Sjögren's syndrome (SSp) is a progressive autoimmune disease characterized by a sicca syndrome but also by the occurrence of systemic manifestations. These manifestations, present in 30–40 % of patients, are varied, can involve many organs, and coexist with each other. They are of highly variable severity, and justify an adaptation of the therapeutic management which can range from a simple symptomatic treatment, to the use of immunosuppressants, biotherapies, combined therapies. The ESSDAI activity score makes it possible, in each organ system, to assess the severity and to stratify the management of these systemic disorders. Due to the wide variety of systemic damage encountered, it is important to offer multidisciplinary care. Despite active research and numerous therapeutic trials in progress or to come, no molecule has obtain agreement for the treatment of pSS. Consequently, the various therapeutic strategies that will be discussed here are essentially based on retrospective studies and expert opinions, and based and recent published recommendations.