BackgroundPrimary Sjögren’s syndrome (pSS) is a rare systemic autoimmune disease with no specific treatment at present. To better assess patient symptoms, we have developed a web application (WebApp) to collect patient symptom intensity on a regular basis.ObjectivesTo measure the daily variability of symptoms using the WebApp. We also evaluated its ease of use.Methods45 consecutive patients with pSS were included in 3 referral centers. Symptoms were assessed during the baseline and 3 month visits. We collected the VAS relating to fatigue, dryness and pain as well as the ESSPRI score. Patients used the WebApp daily for 3 months. The variability of symptoms over time was assessed by the predicted median error. This value was determined using a linear regression model, in order to predict the value at the 3rd month, then this value was compared to the actual value collected at the 3rd month during the clinical visit. The ease of use of the WebApp was assessed using a satisfaction score (SUS score).ResultsOf the 45 patients included, 91.1% were women with an average age of 57 years, and low systemic disease activity (84.4% had an ESSDAI score below 5). The intensity of the symptoms collected during the clinical visits was similar at baseline and at 3 months. The values of the median error for each measurement are between 0.5 and 0.8. The 3-month predicted median error values ranged from 2 to -3. The patients all used the web application for 3 months with good attendance (80% of data completion) and were satisfied with this tool (median SUS score = 90).ConclusionSymptoms of pSS fluctuate from day to day in the majority of patients, making a point measurement imprecise. The developed WebApp is easy to use, and could allow more sensitive detection of the effect of a therapeutic intervention. This tool will soon be evaluated during prospective interventional clinical trials.AcknowledgementsI would like to thanks all people who have helped and were directly or indirectly involved in this study.Disclosure of InterestsNone declared
BackgroundThe diagnosis of primary Sjögren Disease (SjD) is currently based on a combination of clinical, histological and biological findings [1]. Current thinking supports anti-Ro60 antibodies as the most specific serum marker, while the impact of anti-Ro52 remains unclear [2].ObjectivesThe aim of this study was to characterize the clinical, serological, biological, transcriptomic and interferon profiles of SjD patients according to their anti-Ro52 status and discuss the role of anti-Ro52 in the prognosis of SjD.MethodsSjD patients were recruited from the European PRECISESADS (378 patients) [3] and the independant Brittany DIApSS cohorts (160 patients) [4]. Four groups were defined: double negative (Ro52-/Ro60-), isolated anti-Ro52 positive (Ro52+), isolated anti-Ro60 positive (Ro60+), and double positive (Ro52+/Ro60+) patients. Clinical information, disease activity, and biological markers linked to disease severity were evaluated. Transcriptome data on whole blood by RNAseq and Type I and type II interferon signatures [5,6] were analyzed for PRECISESADS SjD patients.ResultsIn both cohorts, arthritis, parotidomegaly, and biological markers (hypergammaglobulinemia, rheumatoid factor and inflammation) [7] were significantly more frequent in the double positive group as compared to other groups. ESSDAI, a score representing systemic activity [8], was also significantly higher in double positive patients compared to the others. Transcriptome analysis demonstrated that anti-Ro52 positivity was associated with a strong interferon pathway activation as the lead cause to explain the clinical associations.ConclusionTaken together, these results suggest that SjD patients with anti-Ro52 positivity adopt a more severe phenotype as compared to their negative counterparts, independently of anti-Ro60 positivity.References[1]Brito-Zerón P, Baldini C, Bootsma H, Bowman SJ, Jonsson R, Mariette X, et al. Sjögren syndrome. Nat Rev Dis Primer. 2016 Jul 7;2(1):1–20.[2]Decker P, Moulinet T, Pontille F, Cravat M, De Carvalho Bittencourt M, Jaussaud R. An updated review of anti-Ro52 (TRIM21) antibodies impact in connective tissue diseases clinical management. Autoimmun Rev. 2022 Mar;21(3):103013.[3]Soret P, Le Dantec C, Desvaux E, Foulquier N, Chassagnol B, Hubert S, et al. A new molecular classification to drive precision treatment strategies in primary Sjögren’s syndrome. Nat Commun. 2021 Jun 10;12(1):3523.[4]Cornec D, Jousse-Joulin S, Pers JO, Marhadour T, Cochener B, Boisramé-Gastrin S, et al. Contribution of salivary gland ultrasonography to the diagnosis of Sjögren’s syndrome: toward new diagnostic criteria? Arthritis Rheum. 2013 Jan;65(1):216–25.[5]Kirou KA, Lee C, George S, Louca K, Papagiannis IG, Peterson MGE, et al. Coordinate overexpression of interferon-alpha-induced genes in systemic lupus erythematosus. Arthritis Rheum. 2004 Dec;50(12):3958–67.[6]Chiche L, Jourde-Chiche N, Whalen E, Presnell S, Gersuk V, Dang K, et al. Modular transcriptional repertoire analyses of adults with systemic lupus erythematosus reveal distinct type I and type II interferon signatures. Arthritis Rheumatol Hoboken NJ. 2014 Jun;66(6):1583–95.[7]Baldini C, Pepe P, Quartuccio L, Priori R, Bartoloni E, Alunno A, et al. Primary Sjögren’s syndrome as a multi-organ disease: impact of the serological profile on the clinical presentation of the disease in a large cohort of Italian patients. Rheumatology. 2014 May 1;53(5):839–44.[8]Brito-Zerón P, Kostov B, Solans R, Fraile G, Suárez-Cuervo C, Casanovas A, et al. Systemic activity and mortality in primary Sjögren syndrome: predicting survival using the EULAR-SS Disease Activity Index (ESSDAI) in 1045 patients. Ann Rheum Dis. 2016 Feb;75(2):348–55.Acknowledgements:NIL.Disclosure of InterestsNone Declared.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.
customersupport@researchsolutions.com
10624 S. Eastern Ave., Ste. A-614
Henderson, NV 89052, USA
Copyright © 2024 scite LLC. All rights reserved.
Made with 💙 for researchers
Part of the Research Solutions Family.