European League Against Rheumatism/European Vasculitis Study Group recommendations for the management of vasculitis

2010 ◽  
Vol 22 (1) ◽  
pp. 49-53 ◽  
Author(s):  
Alexandra Villa-Forte
Keyword(s):  
Study Group ◽  
European League Against Rheumatism ◽  
Group Recommendations ◽  
European League

scholarly journals POS0707 SALIVARY GLAND ULTRASONOGRAPHY AND THE CLINICAL FEATURES USING ESSDAI IN PATIENTS OF EARLY-ONSET VERSUS LATE-ONSET WITH PRIMARY SJÖGREN’S SYNDROME

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 603.1-603
Author(s):  
N. Hashimoto ◽  
S. Uchiyama ◽  
T. Nakazawa ◽  
T. Iwasaki ◽  
T. Hashimoto
Keyword(s):  
Nervous System ◽  
Salivary Gland ◽  
Disease Activity ◽  
Early Onset ◽  
Late Onset ◽  
Primary Sjögren’S Syndrome ◽  
Onset Age ◽  
Primary Sjogren’S Syndrome ◽  
European League Against Rheumatism ◽  
European League

Background:Primary Sjögren’s syndrome (pSS) is a chronic inflammatory autoimmune disease characterized by lymphocyte infiltration in salivary and lacrimal glands. pSS affects primarily middle-aged and elderly patients, although younger age groups may also be involved. However, differences of etiology and pathogenesis between early-onset pSS (EOpSS) and late-onset pSS (LOpSS) are unknown. Recently, standardized outcome tools for measuring disease-specific activity and patients’ reported symptoms have been formulated by the European League Against Rheumatism (EULAR) SS study group: the EULAR SS Disease Activity Index (ESSDAI) for systemic features of pSS [1]. Also, as the new imaging techniques, salivary gland ultrasonography (SGUS) proved valuable for assessing salivary gland involvement in SS and seemed to exhibit good diagnostic properties. In addition, previous studies have demonstrated usefulness of SGUS for the prognostic stratification of patients with pSS [2], [3], [4].Objectives:The aim of this study was to examine the differences of etiology and pathogenesis between EOpSS and LOpSS using ESSDAI and SGUS.Methods:Fifty-six pSS patients who fulfilled the American College of Rheumatology (ACR) / European League Against Rheumatism (EULAR) classification criteria for SS were studied. Based on the disease onset age, all pSS patients were divided into two groups as those with the onset age of 40 years old or younger (EOpSS: n=26) and those with the onset age of older than 65 years old (LOpSS: n=30). The clinical findings were evaluated ESSDAI and OMERACT SGUS score at the first visit to our hospital. The ESSDAI (0–123) proposes the evaluation of 12 domains or organ systems (constitutional, lymphadenopathy, glandular, articular, cutaneous, pulmonary, renal, peripheral nervous system, central nervous system, muscular, hematological and biology). All patients were examined SGUS by a single investigator who was blinded to device (TUS-A300; Canon Medical Systems, Tokyo, Japan) with a linear transducer (7.5-10MHz). The OMERACT SGUS score was used for graded changes in the parenchymal homogeneity of salivary glands: grade 0, normal-appearing salivary gland parenchyma; grade 1, minimal change: mild inhomogeneity without hypo/anechoic areas; grade 2, moderate change: moderate inhomogeneity with focal hypo/anechoic areas; grade 3, severe change: diffuse inhomogeneity with hypo/anechoic areas occupying the entire gland surface [5].Results:The proportions of positive sera of RF, anti-SS-A and anti-SS-B antibodies were not different in the two groups, but the disease activities were higher in the EOpSS than in the LOpSS patients by measuring ESSDAI (7.30 vs 4.23, p=0.008), especially in constitutional domain (1.50 vs 0.60, p=0.03), articular domain (1.54 vs 0.40, p=0.0002) and biological domain (1.35 vs 0.90, p=0.04). No difference in salivary secretion was found between two groups (EOpSS: 8.02 vs LOpSS: 6.31 mL/10min.), but the OMERACT SGUS score was higher in LOpSS than in EOpSS patients (2.00 vs 2.70, p=0.0002).Conclusion:Although serological findings were not different, EOpSS patients had higher disease activity but less severe salivary gland degeneration than that in LOpSS patients, suggesting the pathogenesis of these two groups was different.References:[1]Seror R, et al. Ann Rheum Dis. 2010 Jun;69(6):1103-9.[2]Arthritis Care Res (Hoboken). 2014 Jul;66(7):1102-7.[3]Hammenfors DS, et al. Clin Exp Rheumatol. 2015 Jan-Feb;33(1):56-62.[4]Milic V, et al. PLoS One. 2019 Dec 31;14(12): e0226498.[5]Jousse-Joulin S, et al. Ann Rheum Dis. 2019 Jul;78(7):967-973.Disclosure of Interests:None declared

THU0293 PREDICTORS OF LONG-TERM THERAPY WITH GLUCOCORTICOID IN POLYMYALGIA RHEUMATICA

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 374-375
Author(s):  
A. Aoki ◽  
H. Kobayashi
Keyword(s):  
Polymyalgia Rheumatica ◽  
Clinical Symptoms ◽  
Therapy Group ◽  
Classification Criteria ◽  
Term Therapy ◽  
American College Of Rheumatology ◽  
European League Against Rheumatism ◽  
Long Term Therapy ◽  
European League

Background:Polymyalgia rheumatica (PMR) is a common inflammatory condition of elderly persons. Clinical symptoms respond to low-dose glucocorticoids (GC), but treatment is often required for several years. 2015 EULAR/ACR recommendations1)recommend considering early introduction of methotrexate (MTX) in addition to GC, particularly in patients at a high risk for relapse and/or prolonged therapy. However, risk factors for prolonged therapy are not clear yet.Objectives:We investigated predictive factors which corresponded to the long-term GC therapy.Methods:This was a retrospective study in a single general hospital in Japan. We reviewed the medical records of the Japanese patients with PMR between April 2011 and January 2020. Diagnosis of PMR was based on Bird’s criteria or 2012 EULAR/ACR Classification Criteria2). All patients were treated with prednisolone (PSL), according to the BSR and BHPR guidelines3), for more than 6 months. Patients treated with MTX and accompanied by the giant cell arteritis were excluded from this study. Relapse was defined as the reappearance of symptoms associated with elevated C-reactive protein (CRP) levels in patients receiving GC that required an increase in GC dose. Remission was defined as the absence of clinical symptoms and normal CRP with discontinuation of GC. We compared the clinical findings, laboratory data at baseline and clinical course between those who achieved remission within 2 years (early-remission group) and those who required GC therapy for more than 2 years (long-therapy group). Comparisons between groups were made using Student’s t-test and chi-square test (IBM SSPE statistics version 26). This study was approved by the ethics committee of Tokyo Medical University (T2019-0079).Results:As of January 2020, 89 patients have been treated with PSL for more than 6 months. 50 patients have achieved a remission, 29 were undergoing treatment, and 10 have transferred to other hospitals or died (Table 1). The median time required for the patients to achieve remission was 16 months (Interquartile Range 12-21). After one-year GC therapy, remission was achieved in 14% (11/77), 66% (41/62) after 2-year, 84% (47/56) after 3-year, and 91.0% (49/54) after 4-years. Forty-one patients, who achieved remission within 2 years, were included in the early-remission group. Twenty-one were included in the long therapy group (Table 1). There were no differences in sex, age at onset, body mass index, clinical features, and serum albumin at diagnosis. Serum CRP of long-therapy group was significantly higher than those of the early-remission group (Table 2). Mean relapse times in the full follow-up times were 0.4 in the early-remission group and 3.1 in the long-therapy group. Multivariate logistic regression analysis showed that history of relapse till 6 months was significant predictors of the long-term GC therapy (odds ratio, 6.48; 95%CI 1.44-29.12).Conclusion:The remission rates of our study are lower than those of the previous reports. We have tapered GC gradually according to the BSR and BHPR guidelines3). However, some patients need the long-term therapy for more than 2 years. We might consider additional MTX therapy in patients who experience a relapse during the first six months.References:[1]Dejaco C, et al. 2015 recommendations for the management of polymyalgia rheumatica: a European League against Rheumatism/American College of Rheumatology collaborative initiative. Ann Rheum Dis 2015; 74:1799-1807.[2]Dasgupta B, et al: 2012 provisional classification criteria for polymyalgia rheumatica: a European League against Rheumatism/American College of Rheumatology collaborative initiative. Ann Rheum Dis 2012;71: 484-492.[3]Dasgupta, B, et al. BSR and BHPR guidelines for the management of polymyalgia rheumatica. Rheumatology 2010; 49:186-190.Disclosure of Interests:None declared

European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR) SLE classification criteria item performance

2021 ◽  
pp. annrheumdis-2020-219373
Author(s):  
Martin Aringer ◽  
Ralph Brinks ◽  
Thomas Dörner ◽  
David Daikh ◽  
Marta Mosca ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Metabolic Diseases ◽  
Classification Criteria ◽  
Joint Involvement ◽  
Systemic Lupus ◽  
Entry Criterion ◽  
American College Of Rheumatology ◽  
European League Against Rheumatism ◽  
European League

Background/objectivesThe European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR) 2019 classification criteria for systemic lupus erythematosus system showed high specificity, while attaining also high sensitivity. We hereby analysed the performance of the individual criteria items and their contribution to the overall performance of the criteria.MethodsWe combined the EULAR/ACR derivation and validation cohorts for a total of 1197 systemic lupus erythematosus (SLE) and n=1074 non-SLE patients with a variety of conditions mimicking SLE, such as other autoimmune diseases, and calculated the sensitivity and specificity for antinuclear antibodies (ANA) and the 23 specific criteria items. We also tested performance omitting the EULAR/ACR criteria attribution rule, which defines that items are only counted if not more likely explained by a cause other than SLE.ResultsPositive ANA, the new entry criterion, was 99.5% sensitive, but only 19.4% specific, against a non-SLE population that included other inflammatory rheumatic, infectious, malignant and metabolic diseases. The specific criteria items were highly variable in sensitivity (from 0.42% for delirium and 1.84% for psychosis to 75.6% for antibodies to double-stranded DNA), but their specificity was uniformly high, with low C3 or C4 (83.0%) and leucopenia <4.000/mm³ (83.8%) at the lowest end. Unexplained fever was 95.3% specific in this cohort. Applying the attribution rule improved specificity, particularly for joint involvement.ConclusionsChanging the position of the highly sensitive, non-specific ANA to an entry criterion and the attribution rule resulted in a specificity of >80% for all items, explaining the higher overall specificity of the criteria set.

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