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.

scholarly journals EULAR Sjogren's syndrome disease activity index (ESSDAI): a user guide

RMD Open ◽  
2015 ◽  
Vol 1 (1) ◽  
pp. e000022-e000022 ◽  
Author(s):  
R. Seror ◽  
S. J. Bowman ◽  
P. Brito-Zeron ◽  
E. Theander ◽  
H. Bootsma ◽  
...  
Keyword(s):  
Disease Activity ◽  
Sjögren’S Syndrome ◽  
Sjögren's Syndrome ◽  
Activity Index ◽  
Disease Activity Index ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Sjögren‘S Syndrome

scholarly journals Therapeutic Potential of Anti-Interferon α Vaccination on SjS-Related Features in the MRL/lpr Autoimmune Mouse Model

2021 ◽  
Vol 12 ◽  
Author(s):  
Martin Killian ◽  
Fabien Colaone ◽  
Philippe Haumont ◽  
Carole Nicco ◽  
Olivier Cerles ◽  
...  
Keyword(s):  
Mouse Model ◽  
Disease Activity ◽  
Sjögren’S Syndrome ◽  
Sjögren's Syndrome ◽  
Activity Index ◽  
Vaccination Strategy ◽  
Disease Activity Index ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Sjögren‘S Syndrome

Sjögren’s syndrome (SjS) is a frequent systemic autoimmune disease responsible for a major decrease in patients’ quality of life, potentially leading to life-threatening conditions while facing an unmet therapeutic need. Hence, we assessed the immunogenicity, efficacy, and tolerance of IFN-Kinoid (IFN-K), an anti-IFNα vaccination strategy, in a well-known mouse model of systemic autoimmunity with SjS-like features: MRL/MpJ-Faslpr/lpr (MRL/lpr) mice. Two cohorts (with ISA51 or SWE01 as adjuvants) of 26 female MRL/lpr were divided in parallel groups, “controls” (not treated, PBS and Keyhole Limpet Hemocyanin [KLH] groups) or “IFN-K” and followed up for 122 days. Eight-week-old mice received intra-muscular injections (days 0, 7, 28, 56 and 84) of PBS, KLH or IFN-K, emulsified in the appropriate adjuvant, and blood samples were serially collected. At sacrifice, surviving mice were euthanized and their organs were harvested for histopathological analysis (focus score in salivary/lacrimal glands) and IFN signature evaluation. SjS-like features were monitored. IFN-K induced a disease-modifying polyclonal anti-IFNα antibody response in all treated mice with high IFNα neutralization capacities, type 1 IFN signature’s reduction and disease features’ (ocular and oral sicca syndrome, neuropathy, focus score, glandular production of BAFF) improvement, as reflected by the decrease in Murine Sjögren’s Syndrome Disease Activity Index (MuSSDAI) modelled on EULAR Sjögren’s Syndrome Disease Activity Index (ESSDAI). No adverse effects were observed. We herein report on the strong efficacy of an innovative anti-IFNα vaccination strategy in a mouse model of SjS, paving the way for further clinical development (a phase IIb trial has just been completed in systemic lupus erythematosus with promising results).

Efficacy of rituximab in primary Sjögren's syndrome with peripheral nervous system involvement: results from the AIR registry

2011 ◽  
Vol 71 (1) ◽  
pp. 84-87 ◽  
Author(s):  
A Mekinian ◽  
P Ravaud ◽  
P Y Hatron ◽  
C Larroche ◽  
J Leone ◽  
...  
Keyword(s):  
Nervous System ◽  
Disease Activity ◽  
Sjögren’S Syndrome ◽  
Sjögren's Syndrome ◽  
Activity Index ◽  
Disease Activity Index ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Sjögren‘S Syndrome ◽  
Group 1

ObjectiveTo evaluate rituximab (RTX) in primary Sjögren's syndrome (pSS) with peripheral nervous system (PNS) involvement.MethodsPatients with pSS and PNS involvement who were included in the French AIR registry were analysed.Results17 patients (age 60 years (44–78 years); 14 were female) were analysed.Neurological improvement was noted in 11 patients (65%) at 3 months. Rankin scale decreased from 3 (1–5) to 2 (1–5), 2 (1–5) and 2 (1–6) after 3, 6 and 9 months (p=0.02). European Sjögren's Syndrome Disease Activity Index decreased from 18 (10–44) to 11 (5–20), 11 (5–29) and 12 (5–30) after 3, 6 and 9 months (p<0.05).RTX was effective in neurological involvement in 9/10 patients with vasculitis or cryoglobulinaemia (90%) (group 1) at 3 months and in 2/7 cases (29%) without cryoglobulinaemia and vasculitis (p=0.03). Rankin and European Sjögren's Syndrome Disease Activity Index scales decreased significantly in group 1.ConclusionRTX seems effective in cryoglobulinaemia or vasculitis-related PNS involvement in pSS.

scholarly journals Clinical manifestations including the EULAR Sjögren's Syndrome Disease Activity Index in anti-Ro52/SS-A antibody-seropositive patients with Sjögren's syndrome

2020 ◽  
Author(s):  
Hideki Nakamura ◽  
Shimpei Morimoto ◽  
Toshimasa Shimizu ◽  
Ayuko Takatani ◽  
Shin-ya Nishihata ◽  
...  
Keyword(s):  
Disease Activity ◽  
Sjögren’S Syndrome ◽  
Sjögren's Syndrome ◽  
Activity Index ◽  
Disease Activity Index ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Clinical Factors ◽  
Serum Igg ◽  
Sjögren‘S Syndrome

Abstract Background: Relationship between anti-Ro52/SS-A antibody (anti-Ro52) and clinical manifestation of Sjögren's syndrome (SS) was sporadically described regarding involvement of internal organs.Objectives: To determine the clinical factors relevant to anti-Ro52 with SS.Methods: We conducted a retrospective study of subjects with suspected SS (n=149), patients with rheumatoid arthritis (RA) (n=62), and healthy subjects (n=50). We analyzed components of the American-European Consensus Group (AECG) criteria, Raynaud's phenomenon (RP), anti-centromere antibody (ACA), serum IgG, rheumatoid factor, and the EULAR Sjögren's Syndrome Disease Activity Index (ESSDAI).Results: Among the 149 suspected SS subjects, 115 subjects were classified as having SS. Anti-Ro52 was observed in 70 SS patients (60.9%), of whom 31 patients had markedly elevated anti-Ro52 (>500 U/ml). In the SS group, three patients with anti-Ro60−/anti-Ro52+ and 17 patients without anti-Ro were observed. Compared to ACA, the relevance of positive anti-Ro52 toward positive anti-Ro60 was significantly higher (p<0.05). We observed relevance between the anti-Ro52 concentration and anti-Ro60 that was significantly affected by xerophthalmia, xerostomia, ACA seropositivity, RP, serum IgG level, and RF. The anti-Ro52 concentration well discriminated six clinical factors (ROC AUC >0.75) for ACA seropositivity, ESSDAI score ≥1, and RF or moderately high serum IgG, focus score ≥1, and anti-La/SS-B antibody seropositivity (ROC AUC >0.7). A linear relationship between the ESSDAI score and anti-Ro52 was observed.Conclusion: Significant relevance between anti-Ro52 and anti-Ro60 as well as significant items including the ESSDAI regarding the anti-Ro52 concentration was revealed. Regarding relevance toward anti-Ro60, anti-Ro52 had a higher association than ACA.(246/250 words)

Sign in / Sign up

Export Citation Format

Share Document