EULAR Sjögren's syndrome disease activity index (ESSDAI): a user guide

Séror, Raphaèle; Bowman, Simon; Brito-Zerón, Pilar; Theander, Elke; Bootsma, Hendrika; Tzioufas, Athanasios G.; Gottenberg, Jacques-Éric; Ramos‐Casals, M.; Dörner, Thomas; Ravaud, Philippe; Vitali, Claudio; Mariette, Xavier; Asmussen, Karsten; Jacobsen, Søren; Bartoloni, Elena; Gerli, Roberto; Bijlsma, J. W. J.; Kruize, Aike A.; Bombardieri, Stefano; Bookman, Arthur; Kallenberg, Cees G. M.; Meiners, Petra M.; Brun, Johan G.; Jönsson, Roland; Caporali, Roberto; Carsons, Steven E.; Vita, Salvatore De; Papa, Nicoletta Del; Devauchelle, Valérie; Saraux, Alain; Fauchais, Anne‐Laure; Sibilia, Jean; Hachulla, É.; Illei, Gabor G.; Isenberg, David; Jones, Adrian; Manoussakis, Menelaos N.; Mandl, Thomas; Jacobsson, Lennart; Demoulins, Frederic; Montecucco, Carlomaurizio; Ng, Wan‐Fai; Nishiyama, S.; Omdal, Roald; Parke, Ann L.; Praprotnik, Sonja; Tomsic, Matjia; Price, Elizabeth; Scofield, Hal; Sivils, Kathy L.; Smolen, Josef S.; Laqué, Roser Solans; Steinfeld, Serge; Sutcliffe, Nurhan; Sumida, Takayuki; Valesini, Guido; Valim, Valéria; Vivino, Frederick B.; Vollenweider, Cristina

doi:10.1136/rmdopen-2014-000022

Cited by 291 publications

(183 citation statements)

References 25 publications

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“…ESSDAI information of six patients was not available. Patients with ongoing or past organ involvement fulfilling the criteria of at least low activity in any organ domain of ESSDAI28 were considered as patients with extraglandular manifestations (n=16), while patients without systemic involvement were considered as solely suffering from glandular disease (n=15). Biological activity, indicated by parameters such as complement consumption or hypergammaglobulinemia, and constitutional symptoms were not taken into account for subset defintions.…”

Section: Methodsmentioning

confidence: 99%

SIGLEC1 is a biomarker of disease activity and indicates extraglandular manifestation in primary Sjögren's syndrome

et al. 2016

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ObjectivesTo evaluate the interferon (IFN) biomarkers sialic acid binding Ig like lectin 1 (SIGLEC1, CD169) and IFN-γ-inducible protein-10 (IP-10) in patients with primary Sjögren's syndrome (pSS).Methods31 patients fulfilling the American-European criteria for pSS were included. Disease activity was obtained by EULAR Sjögren's syndrome disease activity index (ESSDAI). SIGLEC1 expression on monocytes was analysed using flow cytometry. IP-10 concentrations were determined using Bioplex human Cytokine 27-plex kit. Spearman rank test (SRT) was used for correlation analysis and Mann-Whitney U (MWU) to test for differences between glandular and extraglandular manifestations.ResultsAn activated IFN system was detected by an upregulation of SIGLEC1 expression in 64.5% and by elevated serum level of IP-10 in 78.9% of our patients with pSS. In a subsequent analysis SIGLEC1 expression was found to be upregulated more frequently in patients with extraglandular manifestations (16/16, 100%) compared to patients with exclusively glandular involvement (4/15, 27%). SIGLEC1 expression could significantly discriminate between these two disease subgroups (p=0.0001, MWU) with a positive predictive value (PPV) of 80% for extraglandular disease. Moreover, the expression correlated with disease activity (p=0.005, r=0.54, SRT). Serum IP-10 levels neither differed significantly between glandular and extraglandular disease nor correlated with ESSDAI.ConclusionsOur results indicate that increased SIGLEC1 expression characterises patients with systemic involvement and high disease activity. Therefore, SIGLEC1 determination might be of value for subset definition, risk stratification and differential therapeutic considerations in pSS.

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Section: Methodsmentioning

confidence: 99%

SIGLEC1 is a biomarker of disease activity and indicates extraglandular manifestation in primary Sjögren's syndrome

et al. 2016

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“…The following clinical symptoms occurred in pSS patients' history: sicca syndrome (100%), labial salivary gland biopsy positivity for focal lymphocytic sialadenitis with focus score ≥ 1(80%), blood cytopenia (anemia or leukopenia) (87%), arthritis (67%), vasculitis (53%), anti-SSA/SSB antibody positivity (100%), low C3/C4 complement level (47%), renal involvement (13%), fever and night sweats (20%), lymphadenopathy (20%), weight loss (33%), myositis (7%), and peripheral neuropathy (20%). The EULAR Sjögren's syndrome disease activity (ESSDAI) scores were calculated at the [19]. The median ESSDAI score was 2 which is consistent with low disease activity.…”

Section: Patientsmentioning

confidence: 99%

The role of B7 family costimulatory molecules and indoleamine 2,3-dioxygenase in primary Sjögren’s syndrome and systemic sclerosis

et al. 2016

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B7 costimulatory molecules are present on antigenpresenting cells (APCs) and influence intracellular expression of indoleamine 2,3-dioxygenase (IDO), a molecule with important immunoregulatory functions. We determined the frequency of activated (CD11b+) monocytes expressing B7-1, B7-2, B7-H1, and B7-H2 molecules, and that of CD3+ and CD4+ T cells expressing the corresponding CD28, CTLA-4, PD-1, and ICOS receptors in peripheral blood samples of 20 healthy adults and 9 SSc and 15 pSS patients using flow cytometry. We also examined the intracellular expression of IDO. The expression of CD28 was lower in both SSc and pSS patients. The frequency of CTLA-4 was increased in pSS. The expression of ICOS, a stimulator of T cell activation, was elevated in pSS, but not in SSc, while that of its corresponding costimulatory molecule, B7-H2, was strongly decreased in SSc compared to controls. The frequency of PD-1 expressing T lymphocytes was decreased in both pSS and SSc. The frequency of IDO-expressing APCs, as well as intracellular IDO content in T cells was higher in pSS than in controls. Our investigation identified a number of differences in B7 costimulation between SSc and pSS patients which may play a role in the distinct pathogenesis and clinical features of these autoimmune disorders.

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“…Background: The useful tool to assessing disease activity in primary Sjögren's syndrome(pSS) is an EULAR Sjögren's syndrome disease activity index (ESS-DAI)[. 1,2 Objectives: The aim of this study was indication of laboratory and clinical factors affecting the ESSDAI in pSS patients. Methods: 75 pSS patients were included; 65 (87%) female, 10 (13%)men; mean age 50 years SD ±15.…”

mentioning

confidence: 99%

THU0365 Potential predictive factors influencing essdai of primary sjÖgren’s syndrome patients

sacute¹,

nacute²,

ska³

et al. 2018

Thursday, 14 June 2018

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BackgroundThe useful tool to assessing disease activity in primary Sjögren’s syndrome(pSS) is an EULAR Sjögren’s syndrome disease activity index (ESSDAI)[.1,2 ObjectivesThe aim of this study was indication of laboratory and clinical factors affecting the ESSDAI in pSS patients.Methods75 pSS patients were included; 65 (87%) female, 10 (13%)men; mean age 50 years SD ±15. The tests included:basic laboratory tests, rheumatoid factor (RF), erythrocyte sedimentation rate, CRP, gammaglobulins serum concentration, C4 and C3 component of complement, antinuclear antibodies (ANA) - IF test HEp-2000, anti-SS-A and anti-SS-B antibodies evaluation with semi-quantitative immunoblotting, standard ELISA assays for serum cytokines levels (BAFF, APRIL, FLT-3L, TNF-β, IL-21) and β2-microglobulin. Biopsy of minor salivary gland with the histopathological evaluation (focus score-FS) and the immunochemistry was also performed with the CD3+, CD4 +, CD19 +, CD21 +, CD35 +cells presence assessment. The Schirmer’s test and ocular staining score (OSS) were performed. The Bioethics Committee aproval was obtained. Statistics: U Mann-Whitney (continuous variables) tests, Spearman correlation coefficient (correlations between quantative variables) with statistical significance set at p<0.05.ResultsESSDAI depends on FS, the presence of CD4 +, CD3 +cells in the minor salivary glands infiltrates, RF and cryoglobulins (p<0.05). The division of pSS subjects into two subgroups (ESSDAI >5;<5) revealed, that the autoantibodies as anti-SS-A and anti-SS-B, influence the severity of the disease (p=0,046; p=0015 respectively). ESSDAI positively correlated with OSS, but not with the Schirmer’s test, other tested cytokines, ESR, CRP and gammaglobulins concentration - yet interestingly ESSDAI correlates negatively with IgG4 (rho=-0,435).ConclusionsThe results confirm, that organ-related complications are influenced by inflammatory activity. This activity is expressed by mononuclear cell infiltrates (FS), which consist primarily of T-lymphocytes (indicators of active and early stage inflammation). However, contrary to other observations,3 no correlation between ESSDAI and cytokines or β2- microglobulin was found. The correlation of ESSDAI with the presence of pSS marker autoantibodies (anti-SS-A, anti SS-B antibodies), as well as with non-specific ones (RF or cryoglobulins), indicates the immunological disease activity and overactivity of B lymphocytes as suspected. The reduction of IgG4 concentration in pSS patients correlating with higher ESSDAI can be associated with breaking the autotolerance and lack of stimulation of IgG4 production. But the role and importance of IgG4 in immunological processes both as an activator of dependent autoimmune diseases and, on the other hand, the marker of induction of immune tolerance requires further research.References[1] Seror R, Bowman SJ, Brito-Zeron P, et al. EULAR Sjögren’s syndrome disease activity index (ESSDAI): a user guide. RMD Open2015;1: e000022.[2] Risselada AP, Kruize AA, Bijlsma JW. Clinical applicability of...

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EULAR Sjögren's syndrome disease activity index (ESSDAI): a user guide

Cited by 291 publications

References 25 publications

SIGLEC1 is a biomarker of disease activity and indicates extraglandular manifestation in primary Sjögren's syndrome

SIGLEC1 is a biomarker of disease activity and indicates extraglandular manifestation in primary Sjögren's syndrome

The role of B7 family costimulatory molecules and indoleamine 2,3-dioxygenase in primary Sjögren’s syndrome and systemic sclerosis

THU0365 Potential predictive factors influencing essdai of primary sjÖgren’s syndrome patients

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