title: How are we treating our systemic patients with primary Sjögren syndrome? Analysis of 1120 patients.
abstract: OBJECTIVE
  To describe how systemic disease is treated in a large cohort of Spanish patients with primary Sjögren syndrome (pSS) in daily practice, focusing on the adequacy of therapies for the level of systemic activity measured by ESSDAI score.
  
  
  METHODS
  By December 2014, our database included 1120 consecutive patients who fulfilled the 2002 classification criteria for SS. Therapeutic schedules were classified into 4 categories: no systemic therapies, hydroxychloroquine (HCQ) and/or low dose glucocorticoids (GCS) (<20mg/day), high dose GCS (>20mg/day) and use of second-line therapies (immunosuppressive agents, intravenous immunoglobulins [IVIG] and/or rituximab [RTX]).
  
  
  RESULTS
  There were 1048 (94%) women and 72 (6%) men , with a mean age at diagnosis of 54 years. The main drug-based therapeutic approaches for systemic pSS during follow-up were HCQ in 282 (25%) patients, GCS in 475 (42%, at doses >20mg/day in 255-23%), immunosuppressive agents in 148 (13%), IVIG in 25 (2%) and RTX in 35 (3%) patients. HCQ was associated with a lower risk of death (adjusted HR of 0.57, 95% 0.34-0.95). We classified 16 (7%) of the 255 patients treated with >20mg GCS and 21/148 (14%) treated with immunosuppressive agents as patients inadequately treated, mainly associated with articular involvement of low/moderate activity.
  
  
  CONCLUSIONS
  The management of pSS should be organ-specific, using low dose GCS in patients with moderate systemic activity, limiting the use of high dose GCS and second-line therapies to refractory or potentially severe scenarios. The use of systemic therapies for dryness, chronic pain or fatigue is not warranted.
authors.affiliation: Sjögren Syndrome Research Group (AGAUR), Laboratory of Autoimmune Diseases Josep Font, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Department of Autoimmune Diseases, ICMiD, Hospital Clínic, Barcelona, Spain.
authors.forename: H
authors.initials: H
authors.lastname: Gheitasi
authors.affiliation: Sjögren Syndrome Research Group (AGAUR), Laboratory of Autoimmune Diseases Josep Font, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Department of Autoimmune Diseases, ICMiD, Hospital Clínic, Barcelona, Spain; Primary Care Research Group, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Primary Care Centre Les Corts, CAPSE, Barcelona, Spain.
authors.forename: B
authors.initials: B
authors.lastname: Kostov
authors.affiliation: Department of Internal Medicine, Hospital Vall d'Hebron, Barcelona, Spain.
authors.forename: R
authors.initials: R
authors.lastname: Solans
authors.affiliation: Department of Internal Medicine, Hospital Ramón y Cajal, Madrid, Spain.
authors.forename: G
authors.initials: G
authors.lastname: Fraile
authors.affiliation: Department of Internal Medicine, Hospital Universitario Central de Asturias, Oviedo, Spain.
authors.forename: C
authors.initials: C
authors.lastname: Suárez-Cuervo
authors.affiliation: Department of Internal Medicine, Hospital Parc Taulí, Sabadell, Spain.
authors.forename: A
authors.initials: A
authors.lastname: Casanovas
authors.affiliation: Department of Internal Medicine, Hospital Son Espases, Palma de Mallorca, Spain.
authors.forename: F J
authors.initials: FJ
authors.lastname: Rascón
authors.affiliation: Department of Internal Medicine, Hospital Joan XXIII, Tarragona, Spain.
authors.forename: R
authors.initials: R
authors.lastname: Qanneta
authors.affiliation: Department of Internal Medicine, Hospital do Meixoeiro, Vigo, Spain.
authors.forename: R
authors.initials: R
authors.lastname: Pérez-Alvarez
authors.affiliation: Department of Internal Medicine, Hospital Infanta Sofía, Madrid, Spain.
authors.forename: M
authors.initials: M
authors.lastname: Ripoll
authors.affiliation: Department of Internal Medicine, Hospital Infanta Leonor, Madrid, Spain.
authors.forename: M
authors.initials: M
authors.lastname: Akasbi
authors.affiliation: Department of Internal Medicine, Hospital Gregorio Marañón, Madrid, Spain.
authors.forename: B
authors.initials: B
authors.lastname: Pinilla
authors.affiliation: Department of Internal Medicine, Hospital Vall d'Hebron, Barcelona, Spain.
authors.forename: J A
authors.initials: JA
authors.lastname: Bosch
authors.affiliation: Department of Internal Medicine, Hospital Ramón y Cajal, Madrid, Spain.
authors.forename: J
authors.initials: J
authors.lastname: Nava-Mateos
authors.affiliation: Department of Internal Medicine, Hospital Universitario Central de Asturias, Oviedo, Spain.
authors.forename: B
authors.initials: B
authors.lastname: Díaz-López
authors.affiliation: Department of Internal Medicine, Hospital Parc Taulí, Sabadell, Spain.
authors.forename: M L
authors.initials: ML
authors.lastname: Morera-Morales
authors.affiliation: Sjögren Syndrome Research Group (AGAUR), Laboratory of Autoimmune Diseases Josep Font, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Department of Autoimmune Diseases, ICMiD, Hospital Clínic, Barcelona, Spain.
authors.forename: S
authors.initials: S
authors.lastname: Retamozo
authors.affiliation: Sjögren Syndrome Research Group (AGAUR), Laboratory of Autoimmune Diseases Josep Font, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Department of Autoimmune Diseases, ICMiD, Hospital Clínic, Barcelona, Spain.
authors.forename: M
authors.initials: M
authors.lastname: Ramos-Casals
authors.affiliation: Sjögren Syndrome Research Group (AGAUR), Laboratory of Autoimmune Diseases Josep Font, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Department of Autoimmune Diseases, ICMiD, Hospital Clínic, Barcelona, Spain. Electronic address: mbrito@clinic.ub.es.
authors.forename: P
authors.initials: P
authors.lastname: Brito-Zerón
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D006886:Hydroxychloroquine / Q000627:therapeutic use; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D010818:Practice Patterns, Physicians'; D000069283:Rituximab / Q000627:therapeutic use; D012859:Sjogren's Syndrome / Q000188:drug therapy*; D013030:Spain; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D005938:Glucocorticoids; D016756:Immunoglobulins, Intravenous; D007166:Immunosuppressive Agents; D000069283:Rituximab; D006886:Hydroxychloroquine
keywords: Glucocorticoids; Hydroxychloroquine; Intravenous immunoglobulins; Rituximab; Sjögren syndrome; Treatment