EULAR Sjögren's syndrome disease activity index: development of a consensus systemic disease activity index for primary Sjögren's syndrome
Raphaèle Seror,Philippe Ravaud,Simon J Bowman,Gabriel Baron,Athanasios G. Tzioufas,Elke Theander,Jacques-Eric Gottenberg,Hendrika Bootsma,Xavier Mariette,Claudio Vitali +9 more
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TLDR
The ESSDAI is a clinical index designed to measure disease activity in patients with primary Sjögren's syndrome and should facilitate clinical research and be helpful as an outcome measure in clinical trials.Abstract:
Objective To develop a disease activity index for patients with primary Sjogren9s syndrome (SS): the European League Against Rheumatism (EULAR) Sjogren9s syndrome disease activity index (ESSDAI). Methods Thirty-nine SS experts participated in an international collaboration, promoted by EULAR, to develop the ESSDAI. Experts identified 12 organ-specific ‘domains’ contributing to disease activity. For each domain, features of disease activity were classified in three or four levels according to their severity. Data abstracted from 96 patients with systemic complications of primary SS were used to generate 702 realistic vignettes for which all possible systemic complications were represented. Using the 0–10 physician global assessment (PhGA) scale, each expert scored the disease activity of five patient profiles and 20 realistic vignettes. Multiple regression modelling, with PhGA used as the dependent variable, was used to estimate the weight of each domain. Results All 12 domains were significantly associated with disease activity in the multivariate model, domain weights ranged from 1 to 6. The ESSDAI scores varied from 2 to 47 and were significantly correlated with PhGA for both real patient profiles and realistic vignettes (r=0.61 and r=0.58, respectively, p Conclusions The ESSDAI is a clinical index designed to measure disease activity in patients with primary SS. Once validated, such a standardised evaluation of primary SS should facilitate clinical research and be helpful as an outcome measure in clinical trials.read more
Citations
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2016 American College of Rheumatology/European League Against Rheumatism classification criteria for primary Sjögren's syndrome A consensus and data-driven methodology involving three international patient cohorts
Caroline H. Shiboski,Stephen Shiboski,Raphaèle Seror,Lindsey A. Criswell,M. Labetoulle,Thomas M. Lietman,Astrid Rasmussen,Hal Scofield,Claudio Vitali,Simon J Bowman,Xavier Mariette +10 more
TL;DR: A single set of data-driven consensus classification criteria for primary Sjögren's syndrome performed well in validation analyses and are well suited as criteria for enrolment in clinical trials.
Journal ArticleDOI
2016 American College of Rheumatology/European League Against Rheumatism Classification Criteria for Primary Sjögren's Syndrome: A Consensus and Data-Driven Methodology Involving Three International Patient Cohorts
Caroline H. Shiboski,Stephen Shiboski,Raphaèle Seror,Lindsey A. Criswell,M. Labetoulle,Thomas M. Lietman,Astrid Rasmussen,Hal Scofield,Claudio Vitali,Simon J Bowman,Xavier Mariette +10 more
TL;DR: To develop and validate an international set of classification criteria for primary Sjögren's syndrome using guidelines from the American College of Rheumatology and the European League Against Rheumatism.
Journal ArticleDOI
Primary Sjögren’s Syndrome
TL;DR: In the absence of trials involving patients with primary Sjögren's syndrome, severe organ manifestations are treated with immunosuppressive agents (including prednisone, methotrexate, mycophenolate sodium, azathioprine, and cyclophosphamide), in accordance with guidelines for systemic lupus erythematosus and other connective‐tissue diseases.
Journal ArticleDOI
Sjögren syndrome
Pilar Brito-Zerón,W. Baldini,Hendrika Bootsma,Simon J. Bowman,Roland Jonsson,Xavier Mariette,Kathy L. Sivils,Elke Theander,Athanasios G. Tzioufas,Manuel Ramos-Casals +9 more
TL;DR: Knowledge of SjS has progressed substantially, but this disease is still characterized by sicca symptoms, the systemic involvement of disease, lymphocytic infiltration to exocrine glands, the presence of anti-Ro/SSA and anti-La/SSB autoantibodies and the increased risk of lymphoma in patients with SJS.
Journal ArticleDOI
EULAR Sjögren's Syndrome Patient Reported Index (ESSPRI): development of a consensus patient index for primary Sjögren's syndrome
Raphaèle Seror,Philippe Ravaud,Xavier Mariette,Xavier Mariette,Hendrika Bootsma,Elke Theander,Arne Hansen,Manel Ramos-Casals,Thomas Doerner,Stefano Bombardieri,Eric Hachulla,Johan G. Brun,Aike A. Kruize,Sonja Praprotnik,Matija Tomšič,Jacques-Eric Gottenberg,Valérie Devauchelle,Salvatore deVita,C. Vollenweider,Thomas Mandl,Athanasios G. Tzioufas,Steven E. Carsons,Alain Saraux,Nurhan Sutcliffe,Claudio Vitali,Simon J Bowman +25 more
TL;DR: The EULAR SS Patient Reported Index is a very simple index designed to measure patients' symptoms in primary SS and has good construct validity and is well correlated with SSI and PROFAD.
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