ANALYSE EPIDEMIOLOGIQUE ET CLINIQUE DES AMYLOSES DIAGNOSTIQUEES AU CHRU DE RENNES (ETUDE PROSPECTIVE DE 1995 A 1999)

RENNES1-BU Santé (352382103) / SudocPARIS-BIUM (751062103) / SudocSudocFranceF

Bone mineral density and trabecular bone score assessment in systemic sclerosis: A cross-sectional study

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Combination of Capillaroscopic and Ultrasonographic Evaluations in Systemic Sclerosis Results of a Cross-Sectional Study

International audienceObjective - To compare microvascular damages on nailfold capillaroscopy (NFC) with macrovascular manifestations evaluated by hand power Doppler ultrasonography (PDUS) in systemic sclerosis (SSc) patients, and to assess the associations of these damages with the main digital manifestations of the disease: digital ulcers, acroosteolysis, and calcinosis.Methods - NFC, hand radiographs, and PDUS were systematically performed in 64 unselected SSc patients. PDUS evaluation with assessment of ulnar artery occlusion (UAO) and finger pulp blood flow (FPBF) were performed blinded for the results of radiographs and NFC.Results - UAO and pathologic FPBF were associated with severe capillary loss (Conclusion - Microvascular damages evaluated by NFC and macrovascular features like UAO assessed by PDUS show concordant associations with the main digital manifestations of the disease.<br

Synovial involvement assessed by power Doppler ultra-sonography in systemic sclerosis results of a cross-sectional study

International audienceObjectives - To characterize hand synovial manifestations assessed by power Doppler ultrasonography (PDUS) in a cohort of unselected patients fulfilling the 2013 ACR/EULAR classification criteria for SSc and to evaluate the associations of these synovial manifestations with the main general clinical and biological features of SSc. Methods - One hundred and three SSc patients were consecutively included and underwent PDUS evaluation of both hands assessing synovial and tenosynovial manifestations according to the OMERACT definitions. Clinical, biological and immunological SSc characteristics were recorded at the same time. Results - Thirty-three patients (32%) had ultrasonographic synovial/tenosynovial involvement. The two main PDUS features were Doppler-positive/inflammatory synovitis (n = 18, 17.5%) and sclerosing tenosynovitis (TS) (n = 19, 18.4%). Inflammatory synovitis was more frequent in the wrist and MCP joints. Sclerosing TS was more frequent in men [odds ratio (OR) = 6.32, 95% CI: 2.17, 18.41; P = 0.001] and was associated with anti-RNA polymerase III antibodies (OR = 10.93, 95% CI: 1.84, 65.12; P = 0.01), diffuse SSc (OR = 18.24, 95% CI: 4.80, 69.32; P 5 mg/l (OR = 5.50, 95% CI: 1.81, 16.70; P = 0.001), pericarditis (OR = 7.81, 95% CI: 1.58, 38.71; P = 0.017) and inflammatory arthralgia (OR = 15.96, 95% CI: 2.80, 91.02; P = 0.002). Inflammatory synovitis and sclerosing TS were not significantly associated within an individual patient (OR = 2.77, 95% CI: 0.88, 8.70; P > 0.05). Conclusions - Ultrasonographic synovial involvement is frequent in patients fulfilling the 2013 ACR/EULAR classification criteria and PDUS may have a part to play in a more accurate and precise description of musculoskeletal manifestations of the disease, especially as the question of a treat-to-target approach is arising for SSc

Impact of Three Different Algorithms for the Screening of SSc-PAH and Comparison with the Decisions of a Multidisciplinary Team

International audienceBackground: to compare three existing screening algorithms of pulmonary arterial hypertension (PAH) in systemic sclerosis (SSc) with the results of a multidisciplinary team (MDT) meeting from a tertiary center. Methods: we conducted a monocentric longitudinal study from 2015 to 2018. All patients with SSc according to LeRoy’s classification were eligible. Patients were excluded in the case of missing data required by any of the three screening algorithms. The algorithms were applied for each patient at inclusion. Right heart catheterization (RHC) was performed based on the MDT decision. MDT members were all blinded from the results of the three algorithms regarding RHC recommendations. The RHC recommendations of each algorithm were compared with the MDT decision, and the impact on diagnosis and management was evaluated. Results: 117 SSc patients were consecutively included in the study, and 99 had follow-up data over the three-year duration of the study (10 deaths). Among the 117 patients, the MDT suggested RHC for 16 patients (14%), DETECT algorithm for 28 (24%), ASIG for 48 (41%) and ESC/ERS 2015 for 20 (17%). Among the 16 patients who had RHC, SSc-PAH was diagnosed in seven. Among patients with an initial recommendation of RHC based on at least one algorithm but not according to the MDT meeting, no SSc-PAH was diagnosed during the three-year follow-up. Results were unchanged when the new 2018 definition of PAH was applied instead of the previous definition. Conclusion: a MDT approach appears interesting for the screening of SSc-PAH, with a significant reduction of RHC performed in comparison with dedicated algorithms. The specific relevance of a MDT for the management and follow-up of patients with RHC recommended by existing algorithms but with no PAH warrants further studies

Efferocytosis capacities of blood monocyte‐derived macrophages in systemic sclerosis

International audienceA defect in the apoptotic cell clearance (efferocytosis) by phagocytic cells may participate in autoimmunity and chronic inflammation. The mechanisms leading to the emergence of autoimmunity in systemic sclerosis (SSc) are still to be determined. In this study, the efferocytosis capacities of blood monocyte‐derived macrophages (MDM) from patients with SSc were evaluated. Blood monocytes obtained from patients with SSc and healthy donors (HD) were differentiated in vitro into macrophages. The capacities of MDM to engulf CFSE+ apoptotic Jurkat human T lymphocytes were compared between SSc MDM and HD using flow cytometry. The expression of classical engulfing receptors in SSc MDM and HD MDM was also evaluated and their involvement in the modulation of efferocytosis was confirmed using a siRNA approach. The mean phagocytic index (PI) reflecting efferocytosis capacities of SSc MDM (PI = 19.3 ± 3.0; n = 21) was significantly decreased in comparison with the PI of HD MDM (PI = 35.9 ± 3.0; n = 31; P < 0.001). In comparison with HD, SSc MDM exhibited a downregulated expression of scavenger receptor (SR)‐B1, SR‐A1 and integrin β5 (ITGβ5). In HD MDM, the extinction of these receptors was followed by a reduction of efferocytosis only for the repression of ITGβ5, suggesting a possible selective role of this integrin in the impaired efferocytosis observed in SSc. As efferocytosis may be at the crossroads of inflammation, autoimmunity and fibrosis, in showing impaired efferocytosis capacities of blood MDM in SSc, our study offers new pathogenesis considerations for the involvement of macrophages in the autoimmune processes driving this disorder

HISTORY OF SILICA DUST EXPOSURES AND ASSOCIATION WITH CHEST HRCT AND CLINICAL CHARACTERISTICS IN SYSTEMIC SCLEROSIS

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The neglected association of crystalline silica exposure and systemic sclerosis

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Silica Exposure and Scleroderma: More Bridges and Collaboration Between Disciplines Are Needed

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The nosology of systemic sclerosis: how lessons from the past offer new challenges in reframing an idiopathic rheumatological disorder

International audienceSystemic sclerosis is a rare connective tissue disease characterised by a wide range of clinical manifestations. Compared with previous sets of criteria, the 2013 American College of Rheumatology and European League Against Rheumatism (ACR/EULAR) classification of systemic sclerosis encompasses a broader and more relevant spectrum of the condition. Nonetheless, clinical and prognostic heterogeneity persists among patients fulfilling these criteria. The next task in the classification of systemic sclerosis is the development of new subset criteria that can successfully identify subgroups of patients with distinct prognostic or pathophysiological features. In this Viewpoint we describe the history of systemic sclerosis over the past century with the objective of highlighting the effect of previous nosological debates on efforts to understand and manage this disorder. Rather than seeking to present a systematic review of possible subgrouping for systemic sclerosis in relation to prognosis, we aim to clarify how nosological considerations have influenced our understanding of the cause and prognosis of this so-called idiopathic rheumatological disorder and how aetiological, prognostic, and pathophysiological hypotheses have helped to describe clusters within the disease. By reflecting on past nosological debates and endeavours, we identify challenges for the current initiative to develop a new subgrouping of systemic sclerosis