title: Immunological profile in primary Sjögren syndrome: clinical significance, prognosis and long-term evolution to other auto-immune disease.
abstract: OBJECTIVE
  To study evolution of pSS immunological profile, impact on pSS activity and the long-term evolution of patients with atypical auto-antibodies in a bicentric cohort of patients with pSS (n=445, mean age 53.6+/-14years, mean follow-up 76.1+/-51months).
  
  
  RESULTS
  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 78months), 6 systemic lupus erythematosus (mean occurrence delay 77months) and 2 systemic sclerosis (mean occurrence delay 133+/-64months). 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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Internal Medicine, Limoges University Hospital, France. anne-laure.fauchais@chu-limoges.fr
authors.forename: A L
authors.initials: AL
authors.lastname: Fauchais
authors.forename: C
authors.initials: C
authors.lastname: Martel
authors.forename: G
authors.initials: G
authors.lastname: Gondran
authors.forename: M
authors.initials: M
authors.lastname: Lambert
authors.forename: D
authors.initials: D
authors.lastname: Launay
authors.forename: M O
authors.initials: MO
authors.lastname: Jauberteau
authors.forename: E
authors.initials: E
authors.lastname: Hachulla
authors.forename: E
authors.initials: E
authors.lastname: Vidal
authors.forename: P Y
authors.initials: PY
authors.lastname: Hatron
mesh_terms: D000974:Antibodies, Antinuclear / Q000032:analysis; D001172:Arthritis, Rheumatoid / Q000150:complications; D005500:Follow-Up Studies; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000150:complications / Q000175:diagnosis / Q000276:immunology; D017285:Polymyositis / Q000150:complications / Q000276:immunology; D011379:Prognosis; D012595:Scleroderma, Systemic / Q000150:complications / Q000276:immunology; D012859:Sjogren's Syndrome / Q000150:complications / Q000175:diagnosis* / Q000453:epidemiology / Q000276:immunology*
chemical_list: D000974:Antibodies, Antinuclear