title: Systemic involvement in primary Sjogren's syndrome evaluated by the EULAR-SS disease activity index: analysis of 921 Spanish patients (GEAS-SS Registry).
abstract: OBJECTIVE
  To evaluate systemic involvement in primary SS in a large cohort of Spanish patients using the EULAR-SS disease activity index (ESSDAI) definitions.
  
  
  METHODS
  Systemic involvement was characterized using ESSDAI definitions for the 10 clinical domains (constitutional, lymphadenopathy, glandular, articular, cutaneous, pulmonary, renal, peripheral nervous system, central nervous system and muscular). ESSDAI scores at diagnosis, during follow-up and cumulated at the last visit were calculated.
  
  
  RESULTS
  The cohort consisted of 921 patients. After a mean follow-up of 75 months, 77 (8%) patients still had an ESSDAI score of zero at the last visit. Organ by organ, the percentage of patients who developed activity during the follow-up (ESSDAI score ≥ 1 at any time) ranged between 1.4% and 56%, with articular, pulmonary and peripheral neurological involvement being the most common. Logistic multivariate regression analysis showed the following features at diagnosis and had the closest association with systemic activity (statistically significant independent variables in at least two domains): cryoglobulinaemia in five domains; anaemia, lymphopenia and low C3 levels in three domains each and age <35 years in two domains. Sicca features, ANA and RF at diagnosis were not associated with a higher cumulated activity score in any clinical domain.
  
  
  CONCLUSIONS
  Primary SS is undeniably a systemic disease, with the joints, lungs, skin and peripheral nerves being the most frequently involved organs. Cytopenias, hypocomplementaemia and cryoglobulinaemia at diagnosis strongly correlated with higher cumulated ESSDAI scores in the clinical domains. Clinically the ESSDAI provides a reliable picture of systemic involvement in primary SS.
authors.affiliation: Servei de Malalties Autoimmunes Sistèmiques, Hospital Clínic, C/Villarroel, 170, 08036 Barcelona, Spain. mramos@clinic.ub.es.
authors.forename: Manuel
authors.initials: M
authors.lastname: Ramos-Casals
authors.forename: Pilar
authors.initials: P
authors.lastname: Brito-Zerón
authors.forename: Roser
authors.initials: R
authors.lastname: Solans
authors.forename: María-Teresa
authors.initials: MT
authors.lastname: Camps
authors.forename: Arnau
authors.initials: A
authors.lastname: Casanovas
authors.forename: Bernardo
authors.initials: B
authors.lastname: Sopeña
authors.forename: Bernardino
authors.initials: B
authors.lastname: Díaz-López
authors.forename: Francisco-Javier
authors.initials: FJ
authors.lastname: Rascón
authors.forename: Rami
authors.initials: R
authors.lastname: Qanneta
authors.forename: Guadalupe
authors.initials: G
authors.lastname: Fraile
authors.forename: Roberto
authors.initials: R
authors.lastname: Pérez-Alvarez
authors.forename: José-Luis
authors.initials: JL
authors.lastname: Callejas
authors.forename: Mar
authors.initials: M
authors.lastname: Ripoll
authors.forename: Blanca
authors.initials: B
authors.lastname: Pinilla
authors.forename: Miriam
authors.initials: M
authors.lastname: Akasbi
authors.forename: Eva
authors.initials: E
authors.lastname: Fonseca
authors.forename: Jesús
authors.initials: J
authors.lastname: Canora
authors.forename: María-Elvira
authors.initials: ME
authors.lastname: Nadal
authors.forename: Gloria
authors.initials: G
authors.lastname: de la Red
authors.forename: Inés
authors.initials: I
authors.lastname: Fernández-Regal
authors.forename: Iratxe
authors.initials: I
authors.lastname: Jiménez-Heredia
authors.forename: Josep-Angel
authors.initials: JA
authors.lastname: Bosch
authors.forename: María-del-Mar
authors.initials: MD
authors.lastname: Ayala
authors.forename: Lluisa
authors.initials: L
authors.lastname: Morera-Morales
authors.forename: Brenda
authors.initials: B
authors.lastname: Maure
authors.forename: Arantxa
authors.initials: A
authors.lastname: Mera
authors.forename: Marc
authors.initials: M
authors.lastname: Ramentol
authors.forename: Soledad
authors.initials: S
authors.lastname: Retamozo
authors.forename: Belchin
authors.initials: B
authors.lastname: Kostov
mesh_terms: D000328:Adult; D000368:Aged; D015331:Cohort Studies; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007592:Joint Diseases / Q000453:epidemiology; D008171:Lung Diseases / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D012042:Registries; D012044:Regression Analysis; D012720:Severity of Illness Index; D012859:Sjogren's Syndrome / Q000175:diagnosis* / Q000453:epidemiology*; D012871:Skin Diseases / Q000453:epidemiology; D013030:Spain / Q000453:epidemiology
keywords: anti-Ro/SS-A antibodies; cryoglobulins; extraglandular involvement; hypocomplementemia; primary Sjögren’s syndrome