Background: Dupilumab is the first biologic available to treat atopic dermatitis (AD). Its effectiveness and safety were demonstrated in clinical trials. Objective:We sought to assess the effectiveness and safety of dupilumab in adults with AD in a real-life French multicenter retrospective cohort.Methods: Patients were included between March 2017 and April 2018. Efficacy outcomes were collected both at baseline and three months (M3), when available, including SCORAD (Scoring Atopic Dermatitis) and EASI (Eczema Area and Severity Index) scores. Adverse events (AE) were recorded at the follow-up. Results:We included 241 patients. The median follow-up time was 3.8±3.7 months. SCORAD75 and EASI75 were achieved in 27/163 (16.6%) and 40/82 (48.8%) patients, respectively. The median SCORAD and EASI at M3 were significantly lower compared with baseline (25±21 vs 56±27.4, p<10 -9 and 4.1±6.8 vs 17.9±15.4, p<10 -9 , respectively).Conjunctivitis was reported in 84/241 (38.2%) patients. The proportion of eosinophilia (>500/mm 3 ) during follow-up (57%) was higher than at baseline (33.7%) (n=172, p<10 -6 ).Dupilumab was stopped in 42 cases, 27 of which were due to an AE. Limitations:No control group, missing data. Conclusion:This real-life study demonstrated results similar to clinical trials, with regard to dupilumab effectiveness, but revealed a higher frequency of conjunctivitis and eosinophilia.

IMPORTANCE In systemic sclerosis (SSc), to date, no study has precisely described the total number and fine distribution of telangiectases (TAs), their clinical association with the disease, and the biological mechanisms causing their development. OBJECTIVES To describe the whole-body distribution of TAs and assess the association between the whole-body TA number and the characteristics of patients with SSc. DESIGN, SETTING, AND PARTICIPANTS A single-center, cross-sectional study was conducted between July 11, 2016, and March 15, 2017, at the National Referral Centre for Rare Systemic and Autoimmune Diseases in France. A population-based sample of 106 adults who fulfilled the 2013 American College of Rheumatology/European League Against Rheumatism criteria for SSc were included; 8 patients who had previously received laser treatment for TAs were excluded. MAIN OUTCOMES AND MEASURES The number of TAs on the whole body (total and those >5 mm) and TA distribution in different areas were recorded. The association with clinical and biological data was studied using univariate and multivariate linear regression. RESULTS A total of 106 patients (83 [78.3%] women) were enrolled, including 12 with precapillary pulmonary hypertension (PH). Mean (SD) age was 60.6 (13.5) years. Telangiectasia distribution was 37.2% on the face, 33.2% on the upper limbs, including 26.4% on the hands, 28.1% on the trunk, including 17.1% for the upper part of the trunk, and 1.5% on the lower limbs. In analysis using the multivariate linear regression model, the whole-body TA number was independently associated with male sex (percentage change, 144.4%; 95% CI, 7.5% to 455.9%; P = .03), PH (162.8%; 95% CI, 5.6% to 553.8%; P = .04), history of pulmonary embolism (336.4%; 95% CI, 39.0% to 1270.1%; P = .01), glomerular filtration rate (−1.6%; 95% CI, −3.2% to −0.1% per 1-mL/min/1.73 m 2 increase; P = .04), and soluble endoglin level (28.2%; 95% CI, 1.2% to 62.5% per 1-ng/mL increase; P = .04). Receiver operating characteristic analyses assessing the ability of TAs to identify the presence of PH revealed that the area under the curve was significant for the TA number on the whole body (0.77; 95% CI, 0.57 to 0.88), on the hands and face (0.81; 95% CI, 0.57 to 0.91), and on the hands (95% CI, 0.77; 95% CI, 0.57 to 0.89). CONCLUSIONS AND RELEVANCE In the patients in this study with SSc, TAs were predominantly located on the face, hands, and the upper part of the trunk. Telangiectases appeared to be associated with vasculopathy features of SSc, particularly with PH and soluble endoglin levels.

BackgroundThere is an ongoing debate regarding the relevance of the 6-minute walking distance (6MWD) in systemic sclerosis (SSc) assessment, widely used as a usual test in these patients as well as an outcome measure in clinical trials. In this work, we aimed to assess the associations between the 6MWD and various disease parameters in patients with SSc.MethodsConsecutive patients followed in our SSc National Reference Centre were included in this cross-sectional study if they fulfilled the 2013 American College of Rheumatology/European League Against Rheumatism criteria for SSc. Data were systematically collected during a comprehensive standardized evaluation that included a 6-minute walk test, clinical assessment, biological results, pulmonary function tests, transthoracic echocardiography, composite scores (European Scleroderma Study Group Activity Index, Medsger severity score, Health Assessment Questionnaire–Disability Index (HAQ-DI)) and treatments.Associations of the 6MWD with various disease parameters were assessed by linear regression in univariate and multivariate analyses.ResultsThe study population comprised 298 patients (females 81%; mean age 58.2 ± 13.3 years; limited cutaneous SSc 82%; interstitial lung disease (ILD) 42%; pulmonary arterial hypertension (PAH) 6%). The 6MWD was significantly and independently associated with gender, age, body mass index, baseline heart rate (HR), HR variation during the test, PAH, history of arterial thrombosis and C-reactive protein levels, as well as with the HAQ-DI score in a sensitivity analysis. Muscle involvement, joint involvement and ILD were not independently associated with the 6MWD.ConclusionsDuring SSc, the 6MWD is independently associated with initial HR and HR variation; with PAH but not ILD, suggesting that pulmonary vasculopathy may have a greater impact than parenchymal involvement on functional limitation; and with global markers of disease activity and patient disability. These results give clinicians further insight into how to interpret the 6MWD in the context of SSc.Electronic supplementary materialThe online version of this article (doi:10.1186/s13075-017-1489-4) contains supplementary material, which is available to authorized users.

IntroductionSoluble markers of B cell activation are interesting diagnostic and prognostic tools in autoimmune diseases. Data in systemic sclerosis (SSc) are scarce and few studies focused on their association with disease characteristics.Methods1. Serum levels of 14 B cell biomarkers (β2-microglobulin, rheumatoid factor (RF), immunoglobulins (Ig) G, IgA, IgM, BAFF, APRIL, soluble (s)TACI, sBCMA sCD21, sCD23, sCD25, sCD27, CXCL13) were measured in SSc patients and healthy controls (HC). 2. Associations between these biomarkers and SSc characteristics were assessed. 3. The pathophysiological relevance of identified associations was explored by studying protein production in B cell culture supernatant.ResultsIn a discovery panel of 80 SSc patients encompassing the broad spectrum of disease manifestations, we observed a higher frequency of RF positivity, and increased levels of β2-microglobulin, IgG and CXCL13 compared with HC. We found significant associations between several biomarkers and SSc characteristics related to disease phenotype, activity and severity. Especially, serum IgG levels were associated with pulmonary hypertension (PH); β2-microglobulin with Nt-pro-BNP and DLCO; and BAFF with peak tricuspid regurgitation velocity (TRV). In a validation cohort of limited cutaneous SSc patients without extensive ILD, we observed lower serum IgG levels, and higher β2-microglobulin, sBCMA, sCD23 and sCD27 levels in patients with pulmonary arterial hypertension (PAH). BAFF levels strongly correlated with Nt-pro-BNP levels, FVC/DLCO ratio and peak TRV in SSc-PAH patients. Cultured SSc B cells showed increased production of various angiogenic factors (angiogenin, angiopoietin-1, VEGFR-1, PDGF-AA, MMP-8, TIMP-1, L-selectin) and decreased production of angiopoietin-2 compared to HC.ConclusionSoluble markers of B cell activation could be relevant tools to assess organ involvements, activity and severity in SSc. Their associations with PAH could plead for a role of B cell activation in the pathogenesis of pulmonary microangiopathy. B cells may contribute to SSc vasculopathy through production of angiogenic mediators.

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Introduction: Global lockdown in the context of the coronavirus disease 2019 (COVID-19) pandemic is an unprecedented experience. We report here the results of an anonymous questionnaire-based survey on the healthcare and control of chronic IMIDs (chronic immune-mediated inflammatory diseases) within the IMMINENT network during the French lockdown (March 17, 2020-May 11, 2020) and the 2-month period following the end of the lockdown (July 11, 2020). Methods: Two anonymous questionnaires were sent by email to 4500 patients who were followed in a university hospital for an IMID in the departments of gastroenterology, rheumatology, dermatology, pneumology, neurology, and internal medicine. Results: A total of 921/4500 (20.46%) responded to the first survey (impact of the lockdown), and 553/4500 (12.28%) to the second (impact at 2-months post-lockdown). Concerning the impact of the lockdown, 420/915 (45.9%) reported affected follow-up. Similarly, after the lockdown, 248/544 (45.6%) declared a negative impact on their follow-up. The repartition by departments of patients’ perception of an altered follow-up during ( P = .72) and at the end of the lockdown ( P = .77) was not statistically different. Our study highlighted the effects of the COVID-19 pandemic and the restriction measures implemented on the self-reported impact felt by patients on the follow-up of their chronic IMIDs without significant differences among all departments. Conclusion: Our study is original by showing that patients, whatever the type of IMID, shared this same negative perception. This transdisciplinary study demonstrated the importance of a collaborative network among all departments.

BackgroundTelangiectasia (TA), one of the diagnostic criteria for systemic sclerosis (SSc), could be a clinical marker for the severity of vasculopathy, including pulmonary hypertension (PH).ObjectivesWe designed a cross-sectional study: (i) to describe the whole-body distribution of TA, (ii) to assess the associations between the whole-body number of TA and the characteristics of patients, (iii) to determine whether the number of TA may be useful to discriminate SSc-PH patients.MethodsPatients were included in the National Referral Centre for Rare Systemic And Autoimmune Diseases if they fulfilled the 2013 ACR/EULAR criteria for SSc. They were excluded if they had received laser treatment. The whole-body number and distribution of TA were recorded at inclusion. The associations were studied using univariate, adjusted and multiple linear regressions.Results106 patients were enrolled, including 12 with PH. The median (interquartile range) number of TA was 30 (82.7). Their distribution was: 37.2% on the face, 33.2% on the upper limbs including 26.4% on the hands, 28.1% on the trunk including 17.1% for the upper part of the trunk, and 1.5% on the lower limbs. Using multivariate linear regression model, the whole-body telangiectasia number was independently associated with male gender (percentage change (95% CI)=+144.4% (7.5; 455.9), p=0.033), pulmonary hypertension (+162.8% (5.6; 553.8), p=0.038), history of pulmonary embolism (+336.4% (39.0; 1270.1), p=0.012), glomerular filtration rate (−1.6% (-3.2; −0.1) per 1 ml/mn/1.73m2 increase, p=0.038) and soluble endoglin (+28.2% (1.2; 62.5) per 1 ng/ml increase, p=0.039). The ROC analyses assessing the ability of telangiectasia to discriminate the presence of pulmonary hypertension revealed that the area under the curve was significant for the telangiectasia number on the whole body (0.77 (0.57; 0.88)), on the hands and face (0.81 (0.57; 0.91)) and on the hands (0.77 (0.57; 0.89)).ConclusionsIn SSc-patients, TA were predominantly located on the face, hands and the upper part of the trunk. They may reflect the vasculopathy of SSc and could represent a clinical biomarker for vascular disease, particularly for PH, one of the most severe vascular complications of the disease.References[1] Johnson SR. Curr Rheumatol Rep2015;17(5):32.[2] Mould TL, et al. Asian Pac J Allergy Immunol2000;18(4):195–200.[3] Robert-Thomson, et al. Asian Pac J Allergy Immunol2002.[4] Shah AA, et al. J Rheumatol2010;37(1):98–104.[5] Hurabielle C, et al. Arthritis Care Res2015October.[6] Zhang S, Xu D, Li M, et al. Telangiectasia as a potential clinical marker of microvascular lesions in systemic sclerosis patients from EUSTAR data in China. Clin Exp Rheumatol2015;33(4Suppl 91):S106–110.Disclosure of InterestNone declared

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