scholarly journals CRESS: improving the assessment of disease activity in Sjögren's syndrome

2021 ◽  
Author(s):  
David A Isenberg
Keyword(s):  
Disease Activity ◽  
Sjögren’S Syndrome ◽  
Sjögren's Syndrome ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Assessment Of Disease Activity ◽  
Sjögren‘S Syndrome

scholarly journals FRI0182 RISK FACTORS ASSOCIATED WITH RENAL INVOLVEMENT IN PRIMARY SJÖGREN’S SYNDROME: DATA FROM THE SPANISH SJÖGRENSER COHORT

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 674.3-675
Author(s):  
J. Narváez ◽  
C. Sánchez-Piedra ◽  
M. Fernandez Castro ◽  
V. Martinez Taboada ◽  
A. Olive ◽  
...  
Keyword(s):  
Risk Factors ◽  
Renal Disease ◽  
Disease Activity ◽  
Sjögren’S Syndrome ◽  
Sjögren's Syndrome ◽  
Renal Involvement ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Primary Sjögren’S Syndrome ◽  
Sjögren‘S Syndrome

Objectives:To investigate the prevalence, risk factors, and effects of primary renal disease on morbidity and mortality in patients with primary Sjögren’s syndrome (pSS).Methods:All patients in the SJÖGRENSER (registry of adult SSp patients of the Spanish Society of Rheumatology, cross-sectional phase) cohort were retrospectively investigated for the presence of clinically significant renal involvement directly related to pSS activity.Results:Of the 437 patients investigated, 39 (9%) presented overt renal involvement during follow-up. Severe renal disease necessitating kidney biopsy was relatively rare (23%).Renal involvement may complicate pSS at any time during the disease course and is associated with severe disease (indicated by higher scores of involvement, activity, and damage), systemic multiorgan involvement, and a higher frequency of lymphoma. Multivariate analysis showed that older age (odds ratio [OR] 1.03, 95% confidence interval [CI] 1.00–1.07), higher European League Against Rheumatism Sjogren’s Syndrome Disease Activity Index scores (OR 1.1, CI 1.03–1.18), serum anti-La/SSB positivity (OR 6.44, CI 1.36–30.37), and non-vasculitic cutaneous involvement (OR 8.64, 1.33–55.90) were independently associated with this complication.Chronic renal failure developed in 23 of 39 patients (59%); only 1 of them progressed to end-stage renal disease necessitating renal replacement therapy. Patients with renal disease showed higher Sjögren’s syndrome disease damage index scores (SSDDI), higher rates of hospitalization due to disease activity and higher rates of clinically relevant comorbidities.Conclusion:Renal involvement is an uncommon complication in pSS that was observed in 9% of patients. Although categorized as a non-negligible comorbidity, this condition shows a favorable prognosis.Disclosure of Interests:None declared

scholarly journals EULAR Sjögren's syndrome disease activity index: development of a consensus systemic disease activity index for primary Sjögren's syndrome

2009 ◽  
Vol 69 (6) ◽  
pp. 1103-1109 ◽  
Author(s):  
Raphaèle Seror ◽  
Philippe Ravaud ◽  
Simon J Bowman ◽  
Gabriel Baron ◽  
Athanasios Tzioufas ◽  
...  
Keyword(s):  
Disease Activity ◽  
Sjögren’S Syndrome ◽  
Sjögren's Syndrome ◽  
Activity Index ◽  
Disease Activity Index ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Systemic Complications ◽  
Patient Profiles ◽  
Sjögren‘S Syndrome

ObjectiveTo develop a disease activity index for patients with primary Sjögren's syndrome (SS): the European League Against Rheumatism (EULAR) Sjögren's syndrome disease activity index (ESSDAI).MethodsThirty-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.ResultsAll 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<0.001). Compared with 57 (59.4%) of the real patient profiles, 468 (66.7%) of the realistic vignettes were considered likely or very likely to be true.ConclusionsThe 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.

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