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Primary Sjögren's syndrome is a systemic autoimmune disease whose clinical spectrum extends from sicca syndrome to systemic involvement (extraglandular manifestations). Recent reports have focused on expanding the clinical characterization and improving the diagnostic and therapeutic management of systemic Sjögren's.The development of the EULAR-SS disease activity index represents a step forward in the evaluation of systemic Sjögren's, and three multicenter studies including more than 2500 European patients have confirmed that primary Sjögren's syndrome is, undeniably, a systemic autoimmune disease. Systemic involvement plays a key role in the prognosis of primary Sjögren's syndrome, and recent studies have focused on cutaneous, pulmonary, renal and neurological disease features. Other studies comparing the two sets of Sjögren's syndrome criteria (American College of Rheumatology vs. American-European Consensus Group) in real-life patients found a moderate level of agreement. Autoantibodies are clues to an early diagnosis, as positivity confirms an autoimmune origin of the sicca syndrome and may appear several years before the disease diagnosis. In patients with a high clinical suspicion and negative results for the standard determination of anti-Ro/SS-A antibodies, specific detection of anti-Ro52/60 antibodies is recommended. Direct and indirect B-cell blocking seems to be the most promising biological pathway in primary Sjögren's syndrome, especially for systemic involvement, although a large controlled trial has failed to demonstrate the efficacy of rituximab for nonsystemic symptomatology (dryness, fatigue and pain).Dryness of the mucosal surfaces is the pivotal, but not only, clinical involvement that characterizes primary Sjögren's syndrome. There is growing interest in and knowledge of the clinical characterization and therapeutic management of systemic Sjögren's.