title: Primary Sjogren's syndrome as a multi-organ disease: impact of the serological profile on the clinical presentation of the disease in a large cohort of Italian patients.
abstract: OBJECTIVE
  The aims of this study were to describe the clinical presentation of primary SS (pSS) in a large cohort of patients by assessing the prevalence of the patient subgroups at high risk for severe extraglandular manifestations and to explore the influence of the patients' serological profile on disease severity and on immunosuppressive drug utilization.
  
  
  METHODS
  Cumulative demographic, clinical, serological, histological and therapeutic data of 1115 pSS patients were retrospectively evaluated. Independent serological markers for glandular and extraglandular disease manifestations were identified by logistic regression.
  
  
  RESULTS
  The cohort included 1115 (1067 female, 48 male) pSS patients. Severe extraglandular manifestations were detectable in 15% of the patients and were represented by active synovitis (11%), axonal sensory-motor neuropathy (2%), severe leucocytopenia (14%), cutaneous vasculitis (6%) and non-Hodgkin's lymphoma (4.5%). We found that low C3/C4, hypergammaglobulinaemia, RF and cryoglobulinaemia were markers of severity for pSS. According to the number of serological variables, the patients were subdivided into three distinct groups: favourable (no serological markers), intermediate (one serological marker) and poor (two or more serological markers). In comparison with the other two patient groups, pSS patients presenting with two or more adverse determinants had a higher frequency of severe visceral disease complications and required more aggressive therapeutic interventions.
  
  
  CONCLUSIONS
  This study confirmed that the prevalence of the pSS high-risk subset for severe systemic manifestations is ∼15%. Serological markers might help in the early identification of patients who are candidates to receive more aggressive treatments.
authors.affiliation: Rheumatology Unit, University of Pisa, Via Roma 67, 560126 Pisa, Italy. chiara.baldini74@gmail.com.
authors.forename: Chiara
authors.initials: C
authors.lastname: Baldini
authors.forename: Pasquale
authors.initials: P
authors.lastname: Pepe
authors.forename: Luca
authors.initials: L
authors.lastname: Quartuccio
authors.forename: Roberta
authors.initials: R
authors.lastname: Priori
authors.forename: Elena
authors.initials: E
authors.lastname: Bartoloni
authors.forename: Alessia
authors.initials: A
authors.lastname: Alunno
authors.forename: Angelica
authors.initials: A
authors.lastname: Gattamelata
authors.forename: Marta
authors.initials: M
authors.lastname: Maset
authors.forename: Mariagrazia
authors.initials: M
authors.lastname: Modesti
authors.forename: Antonio
authors.initials: A
authors.lastname: Tavoni
authors.forename: Salvatore
authors.initials: S
authors.lastname: De Vita
authors.forename: Roberto
authors.initials: R
authors.lastname: Gerli
authors.forename: Guido
authors.initials: G
authors.lastname: Valesini
authors.forename: Stefano
authors.initials: S
authors.lastname: Bombardieri
mesh_terms: D000328:Adult; D000368:Aged; D015415:Biomarkers / Q000097:blood; D015331:Cohort Studies; D015897:Comorbidity; D003176:Complement C3 / Q000378:metabolism; D003181:Complement C4 / Q000378:metabolism; D003430:Cross-Sectional Studies; D003450:Cryoglobulins / Q000378:metabolism; D005260:Female; D006801:Humans; D007558:Italy / Q000453:epidemiology; D007970:Leukopenia / Q000097:blood / Q000453:epidemiology*; D008228:Lymphoma, Non-Hodgkin / Q000097:blood / Q000453:epidemiology*; D008297:Male; D008875:Middle Aged; D009422:Nervous System Diseases / Q000097:blood / Q000453:epidemiology*; D015995:Prevalence; D012189:Retrospective Studies; D012217:Rheumatoid Factor / Q000097:blood; D012307:Risk Factors; D012859:Sjogren's Syndrome / Q000097:blood / Q000453:epidemiology*; D013585:Synovitis / Q000097:blood / Q000453:epidemiology*; D014657:Vasculitis / Q000097:blood / Q000453:epidemiology*; D005719:gamma-Globulins / Q000378:metabolism
chemical_list: D015415:Biomarkers; D003176:Complement C3; D003181:Complement C4; D003450:Cryoglobulins; D005719:gamma-Globulins; D012217:Rheumatoid Factor
keywords: Sjögren’s syndrome; disease severity; epidemiology