[Evolutive forms of primary Sjögren's syndrome]

Primary Sjogrën's syndrome (pSS) could be restricted in 50% of the cases to glandular involvement with a chronic sicca syndrome, with a considerable alteration of quality of life. It could be complicated by systemic involvements, which are responsible of the visceral severity. Thus systemic complications could appear many years after initial pSS diagnosis and justify long-term surveillance. Initial parotid gland enlargement, Raynaud phenomenon, cutaneous vasculitis and immunological abnormalities (anti-SSA and/or SSB positivity, hypergammaglobulinemia, and cryoglobulinemia) are also implicated in systemic complications.