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Objective To describe the clinical efficacy of tixagevimab/cilgavimab in pre-exposure prophylaxis in patients at risk of severe coronavirus disease 2019 (COVID-19) and unresponsive to vaccination (anti-severe acute respiratory syndrome coronavirus 2 antibodies <260 binding antibody units/ml) in rheumatology. Methods In this multicentre observational study we included patients with autoimmune or inflammatory diseases who received pre-exposure prophylaxis with tixagevimab/cilgavimab between December 2021 and August 2022. The endpoint was incidence of COVID-19 and its severity. Results Tixagevimab/cilgavimab was administered to 115 patients with a median age of 62 years [interquartile range (IQR) 52–71], chronic arthritis (n  = 53), connective tissue disease (n  = 38) or vasculitis (n  = 11). The main background immunosuppressants were rituximab (n  = 98), corticosteroids [ n  = 62; median dose 5 mg (95% CI 5–8)] and methotrexate (n  = 48). During a median follow-up of 128 days (IQR 93–173), COVID-19 occurred in 23/115 patients (20%) and the omicron variant was identified for the eight genotyped patients. During the study period, the average weekly incidence was 1071/100 000 inhabitants in Île-de-France vs 588/100 000 in our patients. Patients who received a two-injection regimen had a lower risk of infection than those with a single injection [16/49 (33%) vs 5/64 (8%), P  = 0.0012]. The COVID-19-positive patients did not differ from uninfected patients concerning age, comorbidities, underlying rheumatic disease and immunosuppressants. All COVID-19 cases were non-severe. The tolerance of injections was excellent. Conclusion In a population with autoimmune or inflammatory diseases at risk of severe COVID-19 unresponsive to vaccination, pre-exposure prophylaxis withy tixagevimab/cilgavimab was associated with a lower incidence of COVID-19 and no severe infections. [ABSTRACT FROM AUTHOR]

Objectives To measure the association between SLE remission and scores of patients-reported outcome (PRO) measures. Methods We performed a prospective cohort study of SLE patients with a 2-year follow-up, using Lupus Patient-Reported Outcome (LupusPRO), Lupus Quality of Life (LupusQoL), Systemic Lupus Erythematosus Quality of Life (SLEQOL) and 36-item Short Form (SF-36) questionnaires. Remission was defined as remission off treatment (ROFT) and remission on treatment (RONT) according to the definitions of remission in SLE consensus. Mixed models accounting for repeated measures were used to compare groups as follow: ROFT and RONT vs no remission and lupus low disease activity state (LLDAS) vs no LLDAS. Results A total of 1478 medical visits and 2547 PRO questionnaires were collected during the follow-up from the 336 recruited patients. A between-group difference in PRO scores reaching at least 5 points on a 0–100 scale was obtained in the following domains: lupus symptoms (LLDAS: +5 points on the 0–100 scale, RONT: +9, ROFT: +5), lupus medication (LLDAS: +5, RONT: +8, ROFT: +9), pain vitality (LLDAS: +6, RONT: +9, ROFT: +6) of LupusPRO; role emotional (LLDAS: +5, RONT: +8), role physical (RONT: +7 and ROFT: +7), bodily pain (RONT: +6), mental health (RONT: +5) and social functioning (RONT: +6) of SF-36. In contrast, a between-group difference reaching at least 5 points was not achieved for any of the LupusQoL and SLEQOL domains. Conclusions RONT, ROFT and LLDAS were associated with significant and clinically relevant higher QoL in most PRO domains of the LupusPRO (disease specific) and SF-36 (generic) questionnaires, but not with LupusQoL and SLEQOL disease-specific questionnaires. [ABSTRACT FROM AUTHOR]

Objective To validate the predictive value of the DAS28 γ-glutamyl transferase (DAS28-γGT) for the occurrence of major cardiovascular (CV) events (MACE) in the 'Etude et Suivi des Polyarthrites Indifférenciées Récentes' ESPOIR cohort. Methods Analysis of 13-year outcome from the ESPOIR cohort. RA patients with missing data for baseline γGT activity and those not followed-up to 1 year were excluded. Baseline DAS28-γGT was calculated using the following formula: 0.56*√TJ-28 + 0.28 * √SJ-28 + 2*ln(γGT) + 0.014 * GH. Our primary outcome was the merit of the DAS28-γGT in predicting the occurrence of MACE. Results Among the 696 patients [536 women, mean (s. d.) age of 49 (12) years], 34 MACE were recorded, with a mean time to event of 71 (44) months. Receiver operating characteristic curve analysis indicated that a DAS28-γGT >9.4 had the best sensitivity and specificity for the diagnosis of MACE during the observation period. DAS28-γGT >9.4 was predictive of the occurrence of MACE, with a hazard ratio (HR) of 3.11 (95% CI 1.41, 5.43). Multivariate Cox analyses confirmed higher DAS28-γGT (HR 2.44, 95% CI 1.05, 5.64) together with age (HR 1.04, 95% CI 1.01, 1.07) and diabetes mellitus (HR 4.12, 95% CI 1.55, 10.95) as independent predictors of MACE. There was a dose effect of the DAS28-γGT for MACE-risk prediction, which was in line with the application of the Framingham risk score. Conclusion The DAS28-γGT was identified in this large prospective cohort as an independent predictor of MACE in patients with RA. The DAS28-γGT is a simple and useful tool to evaluate CV risk in routine and warn the clinician about the CV risk burden in patients with RA. [ABSTRACT FROM AUTHOR]

Objectives To assess whether two cluster analysis-based axial SpA (axSpA) endotypes (A for purely axial; B for both axial and peripheral) are stable over time and are associated with different long-term disease outcomes. Methods K-means cluster analysis was performed at each visit (until 5 years) on 584 patients from the DESIR cohort, who completed all planned visits, and validated in 232 consecutive axSpA patients from the BeGiant cohort. Cluster stability overtime was assessed by kappa statistics. A generalized linear mixed-effect analysis was applied to compare outcomes between clusters. Classification and regression tree (CART) analysis was performed to determine a decision rule able to assign a given patient to a definite cluster at onset. Results Both endotypes remained remarkably stable over time. In the DESIR cohort, patients in cluster B showed higher disease activity, worse functional outcome and higher need for anti-rheumatic drugs than patients in cluster A. CART analysis yielded three main clinical features (arthritis, enthesitis and dactylitis) that accurately determined cluster assignment. These results could be replicated in the Be-GIANT cohort. Conclusion Cluster-based axSpA endotypes were reproducible in two different cohorts, stable over time and associated with different long-term outcome. The axSpA endotype with additional peripheral disease manifestations is associated with more severe disease and requires more intensive drug therapy Clinical trial registration clinicaltrials.gov, https://clinicaltrials.gov , NCT01648907. [ABSTRACT FROM AUTHOR]

The article discusses research which examined prognosis, phenotypes, and outcomes associated with early-onset primary Sjögren's syndrome (pSS). Topics explored include the clinical manifestations and characteristics of pSS, the comparison of clinical and biological phenotypes in two groups of patients with pSS, and the European League Against Rheumatism (EULAR) Sjögren's syndrome disease activity index (ESSDAI) scores recorded.

Objective Fatigue is reported in up to 90% of patients with SLE. This study was conducted to identify the determinants associated with fatigue in a large cohort of patients with SLE, as well as to provide a systematic review of the literature. Methods Patients from the Lupus BioBank of the upper Rhein, a large German–French cohort of SLE patients, were included in the FATILUP study if they fulfilled the 1997 ACR criteria for SLE and had Fatigue Scale for Motor and Cognitive Functions scores collected. Multivariate logistic regression analyses were performed to assess the determinants of fatigue and severe fatigue. Results A total of 570 patients were included (89.1% female). The median age was 42 years (interquartile range 25–75: 34–52). The median value of the SAfety of Estrogens in Lupus Erythematosus National Assessment (SELENA)-SLEDAI was 2 (0–4). Fatigue was reported by 386 patients (67.7%) and severe fatigue by 209 (36.7%). In multivariate analyses, fatigue was associated with depression [odds ratio (OR): 4.72 (95% CI: 1.39–16.05), P = 0.01], anxiety [OR: 4.49 (95% CI: 2.60–7.77), P < 0.0001], glucocorticoid treatment [OR: 1.59 (95% CI 1.05–2.41), P = 0.04], SELENA-SLEDAI scores [OR: 1.05 (95% CI: 1.00–1.12) per 1 point increase, P = 0.043] and age at sampling [OR: 1.01 (95% CI: 1.00–1.03) per 1 year increase, P = 0.03]. Severe fatigue was independently associated with anxiety (P < 0.0001), depression (P < 0.0001), glucocorticoid treatment (P = 0.047) and age at sampling (P = 0.03). Conclusion Both fatigue and severe fatigue are common symptoms in SLE, and are strongly associated with depression and anxiety. Disease activity and the use of glucocorticoids were also independently associated with fatigue, although more weakly. [ABSTRACT FROM AUTHOR]

Objective. To investigate the frequency and risk factors of postoperative complications in RA patients treated with abatacept (ABA). Methods. The Orencia RA registry recruited 1012 patients receiving ABA for RA in routine care. Data from patients treated with ABA who underwent surgery were reviewed to describe the frequency of postoperative complications. Characteristics of patients and surgeries with and without complications were compared to identify factors associated with complications. Results. We identified 205 (20.3%) patients who underwent 263 surgeries, including 176 (66.9%) orthopaedic surgeries. Nineteen (7.2%) surgeries, in 19 patients (9.3%), entailed complications, including 7 delayed wound healing (2.7% of surgeries) and 6 surgical site infections (2.3% of surgeries). The median time between the last infusion of ABA and surgery was 5.9 weeks (range: 0.3-12.0 weeks), with no significant difference between patients with and without complications. The median corticosteroids daily dosage was higher in the group with complications [10.0 (6.25-15.0) vs 6.0 (5.0-10.0) mg/day, P = 0.042]. In multivariate analysis, only the duration of ABA treatment was significantly associated with postoperative complications [adjusted odds ratio (aOR) = 0.94 (95% CI: 0.89, 0.99) for each month of treatment], as were orthopaedic surgeries compared with other kinds of surgery [aOR = 4.45 (95% CI: 1.01, 20.2)]. Conclusion. In RA patients treated with ABA, the rate of surgical complications was low: 7.2% and higher in case of orthopaedic procedure and a more recent initiation of ABA. The median time between surgery and the last infusion of ABA was short and did not influence the rate of postoperative complications. [ABSTRACT FROM AUTHOR]

Objective. To investigate whether subfamilies of the RA-specific autoantibodies to human citrullinated fibrinogen (AhFibA) differentially associate with the RA risk factors, HLA-DRB1 shared epitope containing alleles (SE alleles) and cigarette smoking, and thus help to predict the disease outcome. Methods. AhFibA and their anti-α36-50Cit and anti-β60-74Cit subfamilies were assayed by ELISA, at baseline, in the French ESPOIR (Etude et Suivi des Polyarthrites Indifférenciées Récentes) cohort composed of undifferentiated arthritides and RA patients of <6 months' duration. Cigarette smoking, SE alleles' presence, DAS28, HAQ and modified Sharp-van der Heijde Score data were obtained at baseline, and after follow-up. Results. After 3 years, 701 patients were classified as having RA according to the ACR/EULAR 2010 criteria. Among them, 349 (50%), 203 (29%) and 257 (37%) were AhFibA-, anti-α36-50Cit- and anti-β60-74Cit-positive, respectively. The presence and titres of AhFibA and their subfamilies similarly associated with SE alleles, irrespective of their fine specificity, without significant effect of smoking. Neither their presence nor their titre was associated with DAS28 or HAQ. The presence of at least one subfamily was associated with a faster Sharp/van der Heijde score progression, albeit without correlation with the titre. Conclusion. AhFibA and their main subfamilies are similarly associated with SE alleles without additional effect of smoking. Whatever their fine specificity was, their presence (but not their titre) similarly constituted a marker of faster joint destruction. [ABSTRACT FROM AUTHOR]

Objective. Altered energy expenditure may contribute to the nutritional complications of RA, metabolic syndrome (MS) and rheumatoid cachexia (RC). The main aim of this study was to evaluate whether the altered resting energy expenditure (REE) and physical activity (PA)-related energy expenditure (EE) are related to the duration of RA and inflammatory activity and nutritional complications in RA. Methods. Among patients with well-characterized RA (duration, activity: DAS28 ESR), we measured REE by indirect calorimetry, and PA-EE by actimetry (SenseWear Armband). MS was defined according to the International Diabetes Federation criteria and RC from DXA body composition analysis. The relations between the characteristics and nutritional complications, and EE were analysed by linear regression. Results. Fifty-seven patients were included [73% women, age 57(10) years] with a wide range of disease duration: 3.8(3.0) years, and DAS28 ESR: 3.9(1.4). The mean REE was 1486(256) kcal/day, associated with the DAS28 ESR (β= +0.21, P = 0.02 after adjusting for gender and fat free mass). The prevalence of MS and RC was, respectively, 24 and 18%, and they were unrelated to each other. The patients with MS and/or RC had double the longstanding RA score (P < 0.05), twice the homeostasis model assessment of insulin resistance values (P = 0.052) and halved levels of PA (P<0.05 for metabolic equivalent tasks (METs) and number of steps/day). Two modifiable factors were associated with the presence of MS and/or RC: a low level of PA as METs [exp(B) = 0.03, P = 0.009] and the use of glucocorticoids [exp(B) = 4.08, P = 0.046]. Conclusion. Low levels of PA and treatment by glucocorticoids are associated with the nutritional complications of RA, suggesting the potential for therapeutic interventions. [ABSTRACT FROM AUTHOR]

Objective. To study the effect of age on the risk-benefit balance of abatacept in RA. Methods. Data from the French orencia and RA registry, including a 2-year follow-up, were used to compare the effectiveness and safety of abatacept according to age. Results. Among the 1017 patients, 103 were very elderly (≥75 years), 215 elderly (65-74), 406 intermediate aged (50-64) and 293 very young (<50). At baseline, elderly and very elderly patients had longer disease duration, higher CRP levels and higher disease activity. These age groups showed a lower incidence of previous anti-TNF therapy and less common concomitant use of DMARDs, but a similar use of corticoster-oid therapy. After adjusting for disease duration, RF/ACPA positivity, use of DMARDs or corticosteroids and previous anti-TNF treatment, the EULAR response (good or moderate) and the remission rate were not significantly different between the four age groups. At 6 months, the very elderly had a significantly lower likelihood of a good response than the very young (odds ratio = 0.15, 95% CI: 0.03, 0.68). The decrease in DAS28-ESR over the 24-month follow-up period did not differ by age. Increasing age was associated with a higher rate of discontinuation for adverse events, especially severe infections (per 100 patient-years: 1.73 in very young, 4.65 in intermediates, 5.90 in elderly, 10.38 in very elderly; P< 0.001). Conclusion. The effectiveness of abatacept is not affected by age, but the increased rate of side effects, especially infections, in the elderly must be taken into account. [ABSTRACT FROM AUTHOR]

Objective. The aim was to analyse the accuracy of a hand dynamometer connected to a smartphone to assess RA disease activity through the measurement of handgrip strength (HGS). Methods. Eighty-two RA patients participated in this prospective study. Three types of HGS were assessed: power (Po), pinch (Pi) and tripod (T). An interactive mobile application was developed to capture grip measures. A unilinear regression analysis between HGS and DAS28 was performed. A multivariate regression analysis to identify independent variables related to HGS was also conducted. Results. Sixty-three patients (76.8%) were female. Mean age was 61.3 years. At baseline, a negative correlation between the three HGS measures and DAS28 score was found, as follows: Po, r = -0.65 (95% CI: -0.76, -0.51, P< 0.001); Pi, r=-0.42 (95% CI: -0.59, -0.23, P < 0.001); and T, r =-0.47 (95% CI: -0.63, -0.29, P < 0.001). In a longitudinal analysis of 32 patients, a negative correlation between ΔPo grip and ΔDAS28 was found (r =-0.76, 95% CI: -0.88, -0.56). Po grip was independently correlated with male sex (95% CI: 1.49, 4.14, P =0.002), whereas variables inversely correlated with Po grip were disease duration (95% CI: -2.71, -1.34, P=0.03), patient global assessment (95% CI: -2.41, -1.1, P <0.001) and CRP level (95% CI: -3.56, -1.08, P < 0.001). Conclusion. HGS assessed by a hand dynamometer connected to a smartphone represents an innovative health technology solution that could prompt the self-assessment of RA disease activity in an outpatient setting. [ABSTRACT FROM AUTHOR]

Objectives. To investigate gender-attributable differences regarding clinical outcome [disease activity, physical function and quality of life (QoL)] and radiographic damage in patients with AS over time. Methods. Data from the Outcome in AS International Study were used. Disease activity was assessed by the BASDAI, ASDAS and CRP; physical function by BASFI; QoL by the Short Form-36, Ankylosing Spondylitis Quality of Life (ASQoL) score and European Quality Of Life scale; and radiographic damage by the modified Stoke AS Spine Score (mSASSS). Cross-sectional comparative analyses were done at baseline. Next, separate models were created to assess gender-attributable differences on each outcome measure over time using time-adjusted generalized estimating equations. Results. A total of 216 patients [154 (72.3%) males, mean age 43.6 years (S.D. 12.7), symptom duration 20.5 years (S.D. 11.8), mean follow-up duration 8.3 years (S.D. 4.1)] were included. At baseline, male compared with female patients had lower self-reported disease activity (BASDAI 3.2 vs 3.9, P = 0.03) but more radiographic damage (mSASSS 13.8 vs 6.5, P = 0.02). No significant gender-attributable differences in other clinical parameters were found. In multivariable analysis, male gender was significantly associated with a better ASQoL (B=-1.18, 95% CI: -2.17, -0.20, P = 0.02), and in a separate model with a higher mSASSS over time (B= 8.24, 95% CI: 4.38, 12.09, P<0.01). Conclusion. In this prospective cohort study, no gender-attributable differences in disease activity or physical function over time were found. However, radiographic damage was more severe in males. Furthermore, males had a better QoL over time. [ABSTRACT FROM AUTHOR]

Objective. Outcome of JDM is highly heterogeneous. Our objective was to determine clinical and muscle biopsy features associated with poor outcome and response to treatment. Methods. Clinical data and muscle biopsy were obtained from a monocentric cohort of 29 patients. Clinical subgroups were defined by latent class model analysis of initial and follow-up parameters. Myopathological features were analysed using validated scores. Capillary loss was determined on reconstructions of transversal sections and assessed in the different age groups to take into account variations of muscle capillarization during post-natal development. Regression models were used to identify initial predictors of therapeutic response. Results. Two distinct homogeneous subgroups of patients were identified according to clinical severity and pathological findings. The smallest group of patients (7/29) presented with severe JDM. Compared with the other group (22/29), patients had more severe muscle weakness at disease onset, low remission rate at 12 months, frequent subcutaneous limb oedema or gastrointestinal (GI) involvement and higher myopathological scores (capillary dropout, perifascicular necrosis/regeneration, fibres with internal myonuclei and fibrosis subscores). Relevance of capillary dropout to JDM severity was substantiated by agebased analysis, confirming its major role in JDM pathophysiology. Most of these manifestations could be related to vasculopathy (limb oedema, GI involvement, capillary dropout). Furthermore, Childhood Myositis Assessment Scale <34 with either GI involvement or muscle endomysial fibrosis at disease onset were the best predictors of poor response to treatment. Conclusion. Vasculopathy is prominent in severe JDM. Simple criteria can be used at initial evaluation to identify patients requiring a more intensive therapy. [ABSTRACT FROM AUTHOR]

Objectives. This study aimed to estimate the responsiveness to change of a generic [the 36-item Short Form Health Survey (SF-36)] and a specific health-related quality of life questionnaire [the Lupus Quality if Life questionnaire (LupusQoL)] according to SLE patients' self-reported changes in health status. Methods. In a cohort of 185 SLE patients, quality of life (QoL) was measured three times at 3 month intervals by the LupusQoL and SF-36 questionnaires. Anchors for responsiveness were defined by patients' global assessment of disease impact according to changes in a visual analogue scale (VAS), a 7-point Likert scale and a 0-3 scale of five patient-reported symptoms. Mean change and S.D. in worsening and improving patients according to anchors were estimated using mixed models for repeated measures. Standardized response means (SRMs) were calculated in each group. Results. Patients [mean age 39.6 years (S.D. 10.5), mean Safety of Estrogen in Lupus Erythematosus National Assessment-SLEDAI score 2.6 (S.D. 3.5)] answered a total of 515 questionnaires. For the VAS and Likert global anchors, worsening patients showed a significant decrease in all LupusQoL domains except for burden to others, body image and fatigue and all SF-36 domains with low to moderate responsiveness. Improving patients had a significant increase in all LupusQoL domains except for intimate relationship and all SF-36 domains except for physical functioning and global health with low to moderate responsiveness. Regarding similar domains in the SF-36 and LupusQoL, SRMs were higher in LupusQoL domains in improving patients, while SF-36 domains had larger SRMs in worsening patients. Conclusion. Both the SF-36 and LupusQoL were responsive to changes in QoL in SLE patients over a 3 month interval. LupusQoL seems to be more appropriate to measure improvements in QoL. [ABSTRACT FROM AUTHOR]

Objective. The aim of this study was to evaluate whether salivary gland ultrasonography (SGUS) improves the diagnostic performance of the 2012 ACR classification criteria for SS.Methods. We studied a cohort of 101 patients with suspected SS seen at a single centre in Brittany, France. An SGUS echostructure score ≥2 was considered abnormal. The reference standard was a clinical diagnosis of SS made by a group of experts blinded to SGUS findings.Results. SS was diagnosed in 45 patients. Similar proportions of patients with and without SS had an ocular staining score ≥3. Adding RF positivity and ANA titre ≥1:320 as an alternative to anti-SSA/SSB positivity increased the sensitivity of the serological item without modifying specificity compared with using anti-SSA/SSB alone. SGUS was 60.0% sensitive and 87.5% specific for SS. Adding the SGUS score to the ACR criteria increased sensitivity from 64.4% to 84.4% and only slightly decreased specificity, from 91.1% to 89.3%.Conclusion. The diagnostic performance of the ACR classification criteria for SS is notably improved by adding the SGUS score. SGUS should be included in future classification criteria for SS. [ABSTRACT FROM AUTHOR]

Objectives. Although the last international guidelines for aPL recommended determination of IgA aCL and anti-β2glycoprotein I (aβ2GPI) antibodies for the evaluation of APS in the absence of conventional IgG or IgM aCL and aβ2GPI antibodies, the clinical value of these antibodies remains controversial. We evaluated the clinical utility of IgA aPL and of the determination of target domains of aβ2GPI IgA antibodies.Methods. A retrospective analysis was performed on sera from 439 patients referred for routine detection of aPL IgA by in-house ELISA. Sera positive for aβ2GPI IgA were subsequently tested for aβ2GPI domain 1 (D1) and domain 4/5 (D4/5) antibodies using ELISAs.Results. The prevalence of aβ2GPI IgA antibodies was 16% in patients, significantly different from controls (1%, P < 0.0001). These antibodies were associated with clinical contexts related to APS as thrombosis (28.6% vs 15%, P = 0.009) and SLE (42% vs 15%, P < 0.0001). Interestingly, determination of their target domains revealed a significant association between aβ2GPI IgA directed against D4/5 and SLE without thrombosis (66.7 vs 16.7%, P = 0.002). In contrast, aCL IgA were not more prevalent in patients than in controls.Conclusion. Our study confirmed the interest of aβ2GP1 IgA in the exploration of APS and suggests that identification of target domains of aβ2GP1 IgA may be useful in the evaluation of thrombotic risk in SLE patients. [ABSTRACT FROM PUBLISHER]

Objective. To assess whether factors such as inflammation by laboratory tests and MRI differ between early axial SpA with and without radiographic lesions.Methods. Cross-sectional analysis of baseline data from Devenir des Spondylarthropathies Indifferenciées Récentes (DESIR) cohort patients having recent-onset inflammatory back pain and meeting Assessment of SpondyloArthritis international Society criteria. The baseline evaluation included radiographs and MRI of the SI joints (SIJs) and spine. Patients were classified as having radiographic lesions if they had at least one obvious sacroiliitis, grade 2 for at least one vertebral corner or grade 1 for at least two vertebral corners (at the cervical or lumbar level, according to the modified Stoke Ankylosing Spondylitis Spine Score). Associations between baseline characteristics and the presence of radiographic lesions were evaluated by estimating multi-adjusted odd ratios (aORs) and their 95% CIs using a logistic regression model.Results. Of 475 patients, 180 (37.9%) had radiographic lesions. Factors positively associated with radiographic lesions were alcohol use (aOR 2.42; 95% CI 1.31, 4.44; P = 0.005), CRP level (aOR 1.44; 95% CI 1.13, 1.84; P = 0.003) and SIJ inflammation by MRI (aOR 2.25; 95% CI 1.40, 3.60; P = 0.001); negative associations occurred with good NSAID responsiveness (aOR 0.44; 95% CI 0.24, 0.81; P = 0.008); spinal MRI inflammation was associated with radiographic lesions only in smokers (aOR 1.99; 95% CI 1.01, 3.92; P = 0.048).Conclusion. Alcohol use, poor responsiveness to NSAIDs, CRP elevation, SIJ MRI inflammation and spinal MRI inflammation in smokers were independently associated with radiographic lesions in early axial SpA. [ABSTRACT FROM AUTHOR]

Objective. Catastrophic antiphospholipid syndrome (CAPS) is a life-threatening disease caused by the onset of rapidly progressive and widespread small-vessel thromboses in the presence of aPLs. The aim of this study was to examine pregnancy-related CAPS.Methods. Retrospective series of 13 patients with pregnancy-related CAPS with special focus on the follow-up.Results. Eleven patients had known APS and had been treated with low-molecular-weight heparin (n = 10), aspirin (n = 8), oral anticoagulants (n = 1), HCQ (n = 3) and/or steroids (n = 1) during pregnancy. The most frequent manifestations of CAPS were cutaneous (n = 11), hepatic (n = 11), renal (n = 10), cardiac (n = 8) and neurological (n = 5). CAPS usually followed haemolysis, elevated liver enzymes and low platelet count (HELLP) syndrome (n = 12), which was associated with pre-eclampsia (n = 6) or with eclampsia (n = 3). No maternal death was observed. The perinatal mortality of 54% was related to prematurity with a mean gestational age of 26.6 weeks at onset of CAPS or HELLP syndrome. During a mean follow-up of 4.8 years (range 2–8 years), seven new pregnancies occurred in five patients and led to one miscarriage, four successful pregnancies and two HELLP syndrome with pre-eclampsia or eclampsia that occurred at 28 weeks gestation in both cases despite optimal treatment. No relapse of CAPS was observed. Two mothers suddenly died 2.5 and 6 years after CAPS.Conclusion. The occurrence of HELLP syndrome in a patient with APS should raise the suspicion of CAPS in the following days, and anticoagulation should be maintained post-partum or post-abortum. Subsequent pregnancies are at very high risk. [ABSTRACT FROM AUTHOR]

Objectives. Radiological cervical spine involvement in JIA has already been assessed with a large range of prevalence (5–80%), but most studies were performed a long time ago, in symptomatic JIA and without differentiating subsets of JIA. We set out to describe structural cervical spine involvement in young adults with polyarticular JIA (pJIA) regardless of the cervical symptoms and to compare lesions with those observed in adult RA.Methods. All consecutive pJIAs followed in a transition programme were included. Standard radiographs of the cervical spine, hands, feet and hip were analysed by two independent radiologists blinded to the diagnosis. An RA control group (<55 years), matched for sex and disease duration, was recruited.Results. Fifty-seven pJIA and 58 RA patients were included. Radiographs showed cervical lesions in 65% of pJIA and 67% of RA patients. In total, 51% of pJIA with radiographic abnormalities had no clinical symptoms. In pJIA, the most frequent structural lesions were anterior atlantoaxial subluxation (33%), erosion of the odontoid process (19%), C1–C2 arthritis (17%) and apophyseal joint arthritis (16%). Cervical lesions in pJIA were similar to those in RA except for ankylosis and hypotrophia (P < 0.05). The presence of cervical lesions correlated with a more severe disease.Conclusion. Structural cervical spine involvement is common in pJIA persisting into adulthood, frequently asymptomatic and associated with a more severe disease. We suggest that radiographic assessment of the cervical spine should be done systematically at onset of the disease and regularly during its course regardless of clinical symptoms. [ABSTRACT FROM PUBLISHER]

Objective. The increased cardiovascular risk associated with AS is attributable to multiple factors: disease activity, systemic inflammation, traditional risk factors and NSAIDs. This study aimed to investigate the effects of 24 and 52 weeks of TNF-α inhibitor treatment on arterial stiffness and cardiovascular risk factors.Methods. Arterial stiffness was measured using the augmentation index (AIx) and pulse wave velocity (PWV), while other cardiovascular risk factors [lipid profile, blood pressure (BP) and BMI] were collected for active AS patients.Results. In total, 49 patients, comprising 30 men, were included in the study, with a mean age of 46.9 (12.1) years. Of these, 20 (40.8%) patients were current smokers, while 10 were treated for hypertension. Patients had long-standing [11.9 (9.2) years] and active AS, with a high initial BASDAI [55.0 (18.2)]. Regarding treatment, 26 patients received etanercept, 17 adalimumab and 6 infliximab. No changes were observed in PWV and AIx after 6 or 12 months following TNF-α blockade [PWV 6.97 (2.03) m/s, 6.92 (1.81) m/s and 7.10 (1.95) m/s at baseline, 6 months and 1 year, respectively, P = 0.64; AIx 19.5 (13.1%), 20.2 (12.8%), 18.3 (13.5%), respectively, P = 0.87]. Lipid profiles and other cardiovascular risk factors were unchanged. However, BASDAI, BASFI and biological inflammation were significantly improved.Conclusion. Arterial stiffness was not improved after 6 and 12 months of anti-TNF-α therapy. However, treatment decreased biological inflammation and disease activity without causing any changes in lipid profiles and other traditional cardiovascular risk factors. [ABSTRACT FROM PUBLISHER]

Objective. To describe the pseudotumoural presentation of neuro-Behçet's disease (NBD).Methods. We report here the main characteristics, treatment and outcome of 23 patients (5 personal cases and 18 patients from the literature) with a pseudotumoural presentation of NBD. Pseudotumoural NBD patients were compared with 69 consecutive patients, with a classical form of NBD.Results. The median age was 39 (range 27–48 years) years, with a male predominance (65.2%). Clinical features of the pseudotumoural NBD included hemi- or tetra-pyramidal symptoms (n = 20), headache (n = 17), cerebellar syndrome (n = 3), sphincter impotence (n = 3) and pseudobulbar signs (n = 2). CNS imaging showed pseudotumoural lesions mainly in the capsulo-thalamic area (69.6 vs 11.6% for classical NBD; P < 0.01). Histological analysis revealed necrotic lesions with perivascular inflammatory infiltrate without signs of tumoural or infectious lesions. Patients with pseudotumoural NBD had more severe initial disability status (Rankin's score &geq;3 in 65.2 vs 24.7%; P < 0.01) and had a 3 years' longer duration between neurological signs and BD diagnosis (P = 0.01) compared with patients with classical NBD. Treatment consisted of CSs (n = 21, 95.5%) and immunosuppressive agents (n = 10, 35.7%) that led to complete clinical and imaging remission in 60.9% of patients. Two (8.7%) of the 23 patients with pseudotumoural NBD died of bedridden state complications.Conclusion. The pseudotumoural form of NBD is a rare and life-threatening condition. [ABSTRACT FROM PUBLISHER]

Objectives. To investigate the functional status of difficult-to-treat JIA patients, including patients receiving biotherapies, and to correlate functional status to disease activity.Methods. All JIA patients consecutively evaluated in a paediatric rheumatology referral centre (November 2008 to March 2009) were enrolled in an observational cross-sectional study. The Childhood HAQ (CHAQ), physician's assessment of overall disease activity, parent's assessment of well-being and pain, and active and limited joint numbers were measured.Results. We enrolled 95 patients [27% systemic, 29% polyarticular, 22% enthesitis-related arthritis (ERA) and 23% oligoarticular JIA]. Median disease duration was 3.5 years. Treatment included NSAIDs (56%), MTX (23%), CSs (21%) and biologics (45%). Of all patients, 31 and 56%, respectively, had inactive and minimally active disease. The median CHAQ score was 0.375 (range 0–3). Most patients had no or mild functional disability (61%), impaired well-being (63%) or pain (55%); 10% reported severely impaired function and well-being, 19% severe pain. ERA patients reported worse well-being and pain. CHAQ scores correlated with disease activity. Long-lasting disease and biologic treatment were associated with better well-being and pain scores.Conclusion. Despite the high proportion of severe JIA patients in this cohort, CHAQ values are within the lower range of recent reports, probably related to new therapeutic approaches. Impaired function and well-being remain a challenge for at least 10% of the patients. Impaired well-being and pain in ERA patients require further study. The strong correlation between functional status and well-being underlines the importance of improving function to optimize quality of life. [ABSTRACT FROM PUBLISHER]

Objectives. To measure the frequency of and risk factors for rheumatic manifestations after chikungunya virus (CHIKV) infection and to assess their impact on quality of life (QoL).Methods. In a cohort study among 509 cases diagnosed in France, demographic and clinical characteristics were collected at baseline, and QoL status by 36-item short-form health survey (SF-36), a short form of the Arthritis Impact Measurement Scales 2 (AIMS2-SF) and General Health Questionnaire (GHQ-12) at follow-up. SF-36 scores were compared with population norms. Factors associated with QoL were identified in multivariate linear regression models.Results. A total of 391 (77%) patients participated (53.5% female, mean age 50.2 years). Median time from onset at follow-up was 23.4 months. Among 176 recovered patients, a shorter duration of symptoms was observed in younger age groups and male patients. The probability of full recovery at 1 year was 0.39. Those not recovered were older, had more comorbidities and a longer acute stage with joint swelling. Scores of physical and mental components of the SF-36 and GHQ-12 were low. The AIMS2-SF was affected mainly in symptoms, psychological and social dimensions. Recovered patients did not differ significantly from age- and gender-matched population SF-36 norms. Older age (P = 0.01–0.002) was associated with lower SF-36 scores. Other factors associated with lower SF-36, lower GHQ12 scores and higher AIMS2-SF dimensions were lack of recovery (P = 0.017 to <0.0001), presence of comorbidity (P = 0.005 to <0.0001) and a longer duration of acute stage (P = 0.047 to <0.0001).Conclusion. Medical follow-up with special attention to comorbidity providing information on possible chronic symptoms and giving support for potential depression and anxiety are recommended. [ABSTRACT FROM PUBLISHER]

Objective. Scleroderma renal crisis (SRC) is a severe manifestation of SSc, whose prognosis remains severe, despite treatment with angiotensin-converting-enzyme inhibitor and dialysis. This study was undertaken to describe SRC characteristics, prognosis and outcome, and evaluate the responsibility of CSs in its occurrence.Methods. Analysis concerned 91 SSc patients with SRC who were compared with 427 non-SRC–SSc patients taken as controls.Results. Among the 91 SRC patients, 71 (78.0%) had high blood pressure, 53 (58.2%) hypertensive encephalopathy and 51 (56.0%) thrombotic microangiopathy; 64 (70.3%) had received CSs before or concomitantly with SRC vs 156 (36.5%) non-SRC–SSc patients (P < 0.001). Treated SRC patients also received more prednisone 29.3 (28.4) vs 3.6 (9.9) mg than controls (P < 0.001). SRC clinical outcomes were poor: 49 (53.8%) patients required dialysis, which was definitive for 38. Thirty-seven (40.7%) SRC patients died vs 10.8% of the controls (P < 0.001). Death was most frequent among dialysed patients who never recovered renal function (22 vs 2) and 13 never-dialysed SRC patients died.Conclusions. Although SRC prognosis has improved markedly, SRC remains a severe manifestation of SSc, despite treatment with angiotensin-converting enzyme inhibitor and dialysis. CSs contributed significantly to SRC occurrence. [ABSTRACT FROM PUBLISHER]