There are only few studies, regarding primary Sjögren's syndrome (pSS) long-term clinical course. Moreover, it has been often studied in a restricted number of patients, employing different recruitment and diagnostic criteria. During a 10 years follow-up, we longitudinally evaluate clinical course as well as severe complications and mortality rates in 68 patients with pSS, diagnosed according to the Fox's criteria. Patients were divided into 2 groups according to the autoantibodies pattern detected at the diagnosis: anti-Ro and/or La positive and anti-Ro La negative. Glandular manifestations of pSS were distinctively present in the majority of patients already at time of the diagnosis and serological findings remained typically constant during the whole follow-up. Increased IgG, IgA and ESR as well as low C4 serum levels were significantly prevalent in the Ro and/or La positive group. Finally, we did not found any significant increase in the mortality rate of pSS patients in comparison with the general population.