Sjögren primitif et Sjögren associé

Abstract

Sjögren's syndrome (SS) is a systemic autoimmune disease for which diagnosis is difficult and often delayed because of its highly variable clinical presentation. SS may be primary (pSS) or associated with another systemic or organ-specific autoimmune disease. The typical clinical presentation is a dry oculo-buccal syndrome associated with fatigue and joint and muscle pain. Systemic involvement is seen in more than one third of patients, including lymphoma, and may reveal the disease. International consensus criteria have been established to enable a reliable diagnosis to be made, so that follow-up and appropriate therapeutic measures can be proposed as early as possible. These criteria, which take into account clinical, immunological and histological parameters, are easily applicable in everyday practice, and also make it possible to eliminate the many differential diagnoses of a dry syndrome. The association of SS with another systemic autoimmune disease (lupus, rheumatoid arthritis, systemic scleroderma,…) should be investigated because SS and the associated autoimmune disease may mutually modify their clinical, biological and evolutionary profiles. This leads to the evolution of the concept of “secondary” SS towards that of “associated” SS.