title: [Primary Sjögren's syndrome: exocrine and non-exocrine manifestations].
abstract: OBJECTIVE
  to characterize the exocrine and non-exocrine clinical disease manifestations of 74 patients with primary Sjögren's Syndrome (pSS).
  
  
  METHODS
  retrospective study of pSS patients according to the new 2002 American-European criteria, followed in the Hospital Santa Maria, Rheumatology Outpatient Clinics, in the last 25 years (mean follow-up of 7.7 years).
  
  
  RESULTS
  all patients presented surface exocrine disease, especially xerostomia and keratoconjunctivitis sicca. Internal organ exocrine disease was found in 25 patients (33.8%), whilst only one patient (1.4%) developed monoclonal B lymphocyte disease (pulmonary pseudolymphoma). Fifty-five patients (74.3%) also displayed non-exocrine manifestations, in particular inflammatory mediator-induced disease (68.9%). Inflammatory vascular disease was present in 54% of patients and noninflammatory vascular disease in 27%. Autoimmune thyroiditis was found in 5 patients (6.8%).
  
  
  CONCLUSIONS
  the disease is dominated by oral and ocular involvement, while severe organic manifestations are less frequent. This classification model allows a comparison of patients with physiopathological abnormalities and offers the possibility of finding markers of activity/cronicity related to them.
authors.affiliation: Serviço de reumatologia e doenças ósseas metabólicas, Hospital de Santa Maria, Lisboa, Portugal.
authors.forename: Maria da Graça Henriques da Conceição Negrão Sequeira
authors.initials: Mda G
authors.lastname: Muñoz
authors.forename: Walter Carlos Baptista
authors.initials: WC
authors.lastname: Castelão
authors.forename: Fernando Manuel Diamantino
authors.initials: FM
authors.lastname: Saraiva
authors.forename: José Carlos Teixeira da
authors.initials: JC
authors.lastname: Costa
authors.forename: Mário Fernando Oliveira Viana de
authors.initials: MF
authors.lastname: Queiroz