212 patients were SSA positive and 131 were both SSA and SSB positive. During follow-up, SSA antibodies disappear in 8 patients; 2 of them exhibit new systemic complications of pSS. 68 patients had cryoglobulinemia. 52 patients had other anti-nuclear antibodies (ANA) specificities: anti-RNP (n = 12), anti-centromere (n = 14), anti-DNA native (n = 19), anti-Scl70 (n = 3), anti-JO1 (n = 3), anti-Sm (n = 3) and anti-histone (n = 1).
Fourteen patients developed ANA-associated auto-immune disease during the follow-up: 5 polymyositis (mean apparition delay 78 months), 6 systemic lupus erythematosus (mean occurrence delay 77 months) and 2 systemic sclerosis (mean occurrence delay 133 ± 64 months). Among these 14 patients, only three presented atypical-ANA at pSS diagnosis.
Cryoglobulinemia and anti-SSA and SSB antibodies at diagnosis were associated with new systemic involvements.
Cryoglobulinemia and SSA/SSB positivity are associated with systemic activity after diagnosis in pSS. Although atypical ANA are found in 12 % of the cases, long-term evolution to ANA associated auto-immune diseases concerned patients with active immunological profile and extra-glandular manifestations.