Manuel Ramos-Casals1, Pilar Brito-Zerón2, Raphaèle Seror3, Hendrika Bootsma4, Simon J Bowman5, Thomas Dörner6, Jacques-Eric Gottenberg7, Xavier Mariette3, Elke Theander8, Stefano Bombardieri9, Salvatore De Vita10, Thomas Mandl8, Wan-Fai Ng11, Aike Kruize12, Athanasios Tzioufas13, Claudio Vitali. 1. 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. 2. Sjögren Syndrome Research Group (AGAUR), Laboratory of Autoimmune Diseases Josep Font, IDIBAPS, Department of Autoimmune Diseases, ICMiD, Hospital Clínic, Barcelona, Spain. 3. 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. 4. Department of Rheumatology and Clinical Immunology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands. 5. Rheumatology Department, University Hospital Birmingham NHS Foundation Trust, Birmingham, UK. 6. Rheumatology Department, Charité, University Hospital, Berlin, Germany. 7. Department of Rheumatology, EA 4438, Strasbourg University Hospital, Université de Strasbourg, Strasbourg, France. 8. Department of Rheumatology, Skane University Hospital Malmö, Lund University, Sweden. 9. Rheumatology Unit, University of Pisa, Pisa. 10. Clinic of Rheumatology, Department of Medical and Biological Sciences, University Hospital Santa Maria della Misericordia, Udine, Italy. 11. Musculoskeletal Research Group, Institute of Cellular Medicine, Newcastle University, Newcastle upon Tyne, UK. 12. Department of Rheumatology and Clinical Immunology, University Medical Center Utrecht, Utrecht, The Netherlands. 13. Department of Pathophysiology, School of Medicine, University of Athens, Greece and.
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. CONCLUSION: 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.
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. CONCLUSION: 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.
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