title: Characterization of systemic disease in primary Sjögren's syndrome: EULAR-SS Task Force recommendations for articular, cutaneous, pulmonary and renal involvements.
abstract: OBJECTIVE
  To reach a European consensus on the definition and characterization of the main organ-specific extraglandular manifestations in primary SS.
  
  
  METHODS
  The EULAR-SS Task Force Group steering committee agreed to approach SS-related systemic involvement according to the EULAR SS Disease Activity Index (ESSDAI) classification and proposed the preparation of four separate manuscripts: articular, cutaneous, pulmonary and renal ESSDAI involvement; muscular, peripheral nervous system, CNS and haematological ESSDAI involvement; organs not included in the ESSDAI classification; and lymphoproliferative disease. Currently available evidence was obtained by a systematic literature review focused on SS-related systemic features.
  
  
  RESULTS
  The following information was summarized for articular, cutaneous, pulmonary and renal involvement: a clear, consensual definition of the clinical feature, a brief epidemiological description including an estimate of the prevalence reported in the main clinical series and a brief list of the key clinical and diagnostic features that could help physicians clearly identify these features. Unfortunately we found that the body of evidence relied predominantly on information retrieved from individual cases, and the scientific information provided was heterogeneous. The analysis of types of involvement was biased due to the unbalanced reporting of severe cases over non-severe cases, although the main sources of bias were the heterogeneous definitions of organ involvement (or even the lack of definition in some studies) and the heterogeneous diagnostic approach used in studies to investigate involvment of each organ.
  
  
  CONCLUSIONS
  The proposals included in this article are a first step to developing an optimal diagnostic approach to systemic involvement in primary SS and may pave the way for further development of evidence-based diagnostic and therapeutic guidelines.
authors.affiliation: Sjögren Syndrome Research Group (AGAUR), Laboratory of Autoimmune Diseases Josep Font, IDIBAPS, Department of Autoimmune Diseases, ICMiD, Hospital Clínic, Barcelona, Spain, mramos@clinic.ub.es.
authors.forename: Manuel
authors.initials: M
authors.lastname: Ramos-Casals
authors.affiliation: Sjögren Syndrome Research Group (AGAUR), Laboratory of Autoimmune Diseases Josep Font, IDIBAPS, Department of Autoimmune Diseases, ICMiD, Hospital Clínic, Barcelona, Spain.
authors.forename: Pilar
authors.initials: P
authors.lastname: Brito-Zerón
authors.affiliation: Department of Rheumatology, Hôpitaux Universitaires Paris-Sud, Assistance Publique-Hopitaux de Paris, Le Kremlin Bicêtre, Université Paris-Sud 11, UMR 1184, Le Kremlin Bicêtre, Paris, France.
authors.forename: Raphaèle
authors.initials: R
authors.lastname: Seror
authors.affiliation: Department of Rheumatology and Clinical Immunology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.
authors.forename: Hendrika
authors.initials: H
authors.lastname: Bootsma
authors.affiliation: Rheumatology Department, University Hospital Birmingham NHS Foundation Trust, Birmingham, UK.
authors.forename: Simon J
authors.initials: SJ
authors.lastname: Bowman
authors.affiliation: Rheumatology Department, Charité, University Hospital, Berlin, Germany.
authors.forename: Thomas
authors.initials: T
authors.lastname: Dörner
authors.affiliation: Department of Rheumatology, EA 4438, Strasbourg University Hospital, Université de Strasbourg, Strasbourg, France.
authors.forename: Jacques-Eric
authors.initials: JE
authors.lastname: Gottenberg
authors.affiliation: Department of Rheumatology, Hôpitaux Universitaires Paris-Sud, Assistance Publique-Hopitaux de Paris, Le Kremlin Bicêtre, Université Paris-Sud 11, UMR 1184, Le Kremlin Bicêtre, Paris, France.
authors.forename: Xavier
authors.initials: X
authors.lastname: Mariette
authors.affiliation: Department of Rheumatology, Skane University Hospital Malmö, Lund University, Sweden.
authors.forename: Elke
authors.initials: E
authors.lastname: Theander
authors.affiliation: Rheumatology Unit, University of Pisa, Pisa.
authors.forename: Stefano
authors.initials: S
authors.lastname: Bombardieri
authors.affiliation: Clinic of Rheumatology, Department of Medical and Biological Sciences, University Hospital Santa Maria della Misericordia, Udine, Italy.
authors.forename: Salvatore
authors.initials: S
authors.lastname: De Vita
authors.affiliation: Department of Rheumatology, Skane University Hospital Malmö, Lund University, Sweden.
authors.forename: Thomas
authors.initials: T
authors.lastname: Mandl
authors.affiliation: Musculoskeletal Research Group, Institute of Cellular Medicine, Newcastle University, Newcastle upon Tyne, UK.
authors.forename: Wan-Fai
authors.initials: WF
authors.lastname: Ng
authors.affiliation: Department of Rheumatology and Clinical Immunology, University Medical Center Utrecht, Utrecht, The Netherlands.
authors.forename: Aike
authors.initials: A
authors.lastname: Kruize
authors.affiliation: Department of Pathophysiology, School of Medicine, University of Athens, Greece and.
authors.forename: Athanasios
authors.initials: A
authors.lastname: Tzioufas
authors.forename: Claudio
authors.initials: C
authors.lastname: Vitali
mesh_terms: D000328:Adult; D026683:Advisory Committees; D005060:Europe / Q000453:epidemiology; D019317:Evidence-Based Medicine / Q000379:methods; D005260:Female; D006801:Humans; D007592:Joint Diseases / Q000175:diagnosis / Q000453:epidemiology; D007674:Kidney Diseases / Q000175:diagnosis / Q000453:epidemiology; D008171:Lung Diseases / Q000175:diagnosis / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D015995:Prevalence; D011379:Prognosis; D012720:Severity of Illness Index; D012859:Sjogren's Syndrome / Q000175:diagnosis* / Q000453:epidemiology; D012871:Skin Diseases / Q000175:diagnosis / Q000453:epidemiology
keywords: annular erythema; arthritis; bronchial disease; extraglandular features; glomerulonephritis; interstitial lung disease; interstitial nephritis; primary Sjögren’s syndrome; tubular renal acidosis; vasculitis