AB0510 The relationship between nailfold capillaroscopic assessment and telangiectasia score with severity of peripheral vascular involvement in systemic sclerosis

2013 ◽  
Vol 72 (Suppl 3) ◽  
pp. A944.3-A945
Author(s):  
Y. Yalcinkaya ◽  
O. Pehlivan ◽  
N. Alpay ◽  
A. Omma ◽  
B. Erer ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Vascular Involvement ◽  
Peripheral Vascular ◽  
The Relationship

scholarly journals AB0580 GENDER DIFFERENCES IN SYSTEMIC SCLEROSIS- IMPACT ON DISEASE PHENOTYPE AND PROGNOSIS

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1586.1-1587
Author(s):  
L. Groseanu ◽  
A. Balanescu ◽  
V. Bojinca ◽  
D. Opris-Belinski ◽  
I. Saulescu ◽  
...  
Keyword(s):  
Gender Differences ◽  
Systemic Sclerosis ◽  
Disease Duration ◽  
Vascular Involvement ◽  
Scleroderma Renal Crisis ◽  
Peripheral Vascular ◽  
Male Patients ◽  
Male Sex ◽  
End Stage ◽  
Renal Crisis

Background:The low overall prevalence of systemic sclerosis (SSc) and the low proportion of male patients have resulted in a scarcity of studies assessing sex differences in SSc patients, and contradictory results.Objectives:To evaluated sex influence on disease characteristics at baseline and then to estimate the effects of sex on disease progression and survival.Methods:We performed a retrospective observational study using data extract from the EULAR scleroderma trials and research (EUSTAR) cohort 096. 173 patients were analysed (26 males).The severity of organ system involvement was defined as described previously (1).Results:Males were significantly older at symptom onset (p=0.007) and at first center visit (p=0.009). There were no differences regarding disease duration at first visit or the interval between the onset of Raynaud syndrome and other non-Raynaud manifestations (p=0.06). Male patients were significantly more likely to have ever smoked (p<0.001), males more often had severe or end-stage peripheral vascular involvement (p=0.01). Modified Rodnan skin score (mRSS) was significantly higher in males (p=0.004). We found no difference regarding musculoarticular involvement, except for digital contractures (p=0.001) and tendon friction rubs (p=0,044). Males more often had interstitial lung disease (ILD) (p=0.013) which was also more frequently severe or end-stage (p = 0.003). Cardiac involvement was more common in males: pulmonary hypertension (PAH) (p = 0.018), arrhytmias (p=0.012), left ventricle ejection fraction<45% (p=0.014). The frequency of scleroderma renal crisis (SRC) was higher in males (p=0.025). Gastrointestinal involvement did not differ between groups EScSG (European Scleroderma Study Group) disease activity scores were higher in males (p=0.001). The isolated presence of antitopoisomerase-1 or anticentromere antibodies did not differ between groups. Mortality rate was similar between sexes, although male sex is a independent predictor for the death associated with ILD, SRC, arrythmiasIn multivariate analysis, male sex was independently associated with a higher risk of diffuse cutaneous subtype (OR: 1.56, (1.35 to 1.84); p<0.001), a higher frequency of severe vascular disease (OR: 1.38 (1.11 to 1.67); p<0.001), severe digital contractures (OR:1.92(1.68 to 2,42); p<0.001), interstitial lung disease OR: 1.22 (0.9 to 1.47); p<0.001), severe heart involvement (OR: 1.56 (1.22 to 2,1); p<0.001) and SRC (OR: 3.31 (1.87 to 5620); p<0.003). In the longitudinal analysis, after a mean follow-up of 7.2 (±2.6) years, male sex was predictive of new onset of scleroderma renal crisis (HR: 3.66 (1.82 to 4.86); p=0.006) and heart failure (HR: 1.9 (1.36 to 3.18); p=0.01).Conclusion:In essence, the disease prophyle in females is that of younger age of onset, longer disease duration at first center visit, less severe peripheral vascular involvement, the most frequent cause of death being PAH. In contrast, males are older at onset, present earlier in their disease, have dcSSc, more severe peripheral vascular disease, higher mRSS, more frequent and severe ILD, more frequent heart involvement, higher risk of PAH and SRC, the most common cause of death being ILD. These results raise the point of including sex in the management and the decision-making process.References:[1]Peoples C, Medsger TA Jr, Lucas M et al Gender differences in systemic sclerosis: relationship to clinical features, serologic status and outcomes.J Scleroderma Relat Disord. 2016;1(2):177–240Disclosure of Interests:Laura Groseanu Speakers bureau: novartis, eli-lilly, ucb, pfizer,sandoz, Andra Balanescu Consultant of: pfizer, Speakers bureau: Abbvie, Eli-Lilly, Novartis, Pfizer, Roche, Sandoz, UCB, Violeta Bojinca Speakers bureau: Eli-Lilly, Novartis, Pfizer, Daniela Opris-Belinski Speakers bureau: Abbvie, Eli-Lilly, Novartis, Pfizer, Roche, Ioana Saulescu Speakers bureau: Eli-Lilly, Pfizer, Diana Mazilu: None declared, Sanziana Daia-Iliescu Speakers bureau: sandoz, Andreea Borangiu: None declared, Florian Berghea Paid instructor for: abbvie, Speakers bureau: gideon richter, egis, novartis,ucb, cosmin-laurentiu constantinescu: None declared, CLAUDIA COBILINSCHI Speakers bureau: novartis, Maria Magdalena Negru: None declared, mihai abobului Speakers bureau: gideon richter, Ruxandra Ionescu Consultant of: Consulting fees from Abbvie, Eli-Lilly, Novartis, Pfizer, Roche, Sandoz, Speakers bureau: Consulting and speaker fees from Abbvie, Eli-Lilly, Novartis, Pfizer, Roche, Sandoz

scholarly journals THU0349 THE RELATIONSHIP BETWEEN DISEASE ACTIVITY AND SEVERITY IN SYSTEMIC SCLEROSIS: A PROSPECTIVE ANALYSIS OF 278 PATIENTS

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 406.2-406
Author(s):  
Y. Yalçinkaya ◽  
N. Aliyeva ◽  
S. Amikishiyev ◽  
Y. Cagatay ◽  
B. Artim-Esen ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Disease Activity ◽  
Severe Disease ◽  
Inactive Disease ◽  
Prospective Analysis ◽  
Peripheral Vascular ◽  
Cutaneous Involvement ◽  
Severity Scores ◽  
The Relationship ◽  
Active Disease

Background:Evaluating disease activity and severity in systemic sclerosis (SSc) is crucial to define the patients who are candidate for treatment options.Objectives:We aimed to investigate the relationship between disease activity and severity in SSc in a large cohort.Methods:This is a cross-sectional prospective analysis of 278 (253 females) patients fulfilling ACR/EULAR (2013) classification criteria for SSc. Disease activity and severity were calculated seperately for cutaneous subsets (EscSG and Medsger). The patients were grouped as inactive if EscSG score=0, mildly active if EscSG score>0<3, active if EscSG score≥3.Results:The mean age, duration of Raynaud’s and non-Raynaud features were 48.5±13.1, 12.1±9.8 and 8.3±7.5 years respectively. Characteristics of the SSc patients were summarized in table-1.Ninety-three (34%) and 151(54%) patients were evaluated as having active and mildly active disease. Only 34(12%) patients had inactive disease. The patients with diffuse cutaneous involvement (dcSSc) who were active had higher modified Rodnan Skin score(mRSS) and severity scores of general, skin and joint-tendon involvements; the patients who had mildly active disease also had higher scores of mRSS and severity scores of skin compared to those with inactive disease (table-2).The patients with limited cutaneous involvement (lcSSc) who were assessed as having active disease had higher mRSS and higher severity scores of general, skin, peripheral vascular, lung, joint-tendon and gastrointestinal involvements; the patients who had mildly active disease also had higher scores of mRSS and severity scores of skin, lung, joint-tendon and gastrointestinal involvements compared to those with inactive disease (table-2).Conclusion:One third of our cohort was found to have active disease despite treatment and only 12% had inactive disease. Skin involvement and severity of different organs were shown to be higher in patients with active disease in both cutaneous subsets, together with severity of lung, peripheral vascular and gastrointestinal involvements in active lcSSc. LcSSc and dcSSc patients who had mildly active disease also had severe disease similar to those with active patients. Disease activity and severity should be assessed as separate measurements to highlight the course of the disease and may guide to the management of patients with SSc.Disclosure of Interests:None declared

Anti-endothelial cell antibodies in systemic sclerosis

2010 ◽  
Vol 69 (2) ◽  
pp. 319-324 ◽  
Author(s):  
C Mihai ◽  
J W C Tervaert
Keyword(s):  
Systemic Sclerosis ◽  
Endothelial Cell ◽  
Autoimmune Diseases ◽  
Detection Method ◽  
Vascular Involvement ◽  
Peripheral Vascular ◽  
Serum Samples ◽  
Systemic Autoimmune Diseases ◽  
Clinical Associations ◽  
Heterogeneous Class

Anti-endothelial cell antibodies (AECA) are a heterogeneous class of antibodies whose role in the pathogenesis of autoimmune diseases with vascular involvement has been extensively studied. Systemic sclerosis (SSc) is one of the systemic autoimmune diseases in which endothelial dysfunction is well defined and important in the development of the disease. AECA are present in the serum samples of many patients with SSc. Depending on the detection method and on patient selection, 22–86% of patients test positive for AECA. Among the demonstrated clinical associations, lung and peripheral vascular involvement are the most common. In this paper, the methods of detection, various molecular specificities and the possible pathogenic mechanisms of AECA in SSc are reviewed.

scholarly journals Reassessing the Role of the Active TGF-β1 as a Biomarker in Systemic Sclerosis: Association of Serum Levels with Clinical Manifestations

2016 ◽  
Vol 2016 ◽  
pp. 1-6 ◽  
Author(s):  
Andréa Tavares Dantas ◽  
Sayonara Maria Calado Gonçalves ◽  
Anderson Rodrigues de Almeida ◽  
Rafaela Silva Guimarães Gonçalves ◽  
Maria Clara Pinheiro Duarte Sampaio ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Clinical Manifestations ◽  
Serum Levels ◽  
Vascular Involvement ◽  
Digital Ulcers ◽  
Serum Samples ◽  
Peripheral Blood Mononuclear ◽  
Pbmc Culture ◽  
Significant Difference ◽  
Skin Biopsies

Objective. To determine active TGF-β1 (aTGF-β1) levels in serum, skin, and peripheral blood mononuclear cell (PBMC) culture supernatants and to understand their associations with clinical parameters in systemic sclerosis (SSc) patients.Methods. We evaluated serum samples from 56 SSc patients and 24 healthy controls (HC). In 20 SSc patients, we quantified spontaneous or anti-CD3/CD28 stimulated production of aTGF-β1 by PBMC. The aTGF-β1 levels were measured by ELISA. Skin biopsies were obtained from 13 SSc patients and six HC, and TGFB1 expression was analyzed by RT-PCR.Results. TGF-β1 serum levels were significantly higher in SSc patients than in HC (p< 0.0001). Patients with increased TGF-β1 serum levels were more likely to have diffuse subset (p= 0.02), digital ulcers (p= 0.02), lung fibrosis (p< 0.0001), positive antitopoisomerase I (p= 0.03), and higher modified Rodnan score (p= 0.046). Most of our culture supernatant samples had undetectable levels of TGF-β1. No significant difference in TGFB1 expression was observed in the SSc skin compared with HC skin.Conclusion. Raised active TGF-β1 serum levels and their association with clinical manifestations in scleroderma patients suggest that this cytokine could be a marker of fibrotic and vascular involvement in SSc.

scholarly journals Systemic sclerosis and microscopic polyangiitis after systemic exposure to silicone

2021 ◽  
Author(s):  
Claudia Carrera Muñoz ◽  
Jorge González Rodríguez ◽  
Annabel Abó Rivera ◽  
Elena Estarán ◽  
Jordi Roig Cárcel ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Autoimmune Diseases ◽  
Microscopic Polyangiitis ◽  
Systemic Exposure ◽  
Breast Implants ◽  
Inflammatory Syndrome ◽  
The Relationship

Abstract The relationship between silicon breast implants (SBIs) and autoimmune/inflammatory syndrome induced by adjuvants (ASIA) has been extensively analysed, with discordant results. We present a 45-year-old woman with confirmed systemic exposure to SBI who developed systemic sclerosis (SSc) followed by anti-neutrophil cytoplasmic antibody anti-myeloperoxidase vasculitis with renopulmonary syndrome. The novelty of our case is, first, confirmation of systemic exposure to SBI and, second, chronologic development of not one, but two severe autoimmune diseases. Controversy may still remain regarding SBIs and ASIA because it is unclear that previous studies confirmed systemic exposure to silicon in their cohort of patients.

Characterization and clinical implications of peripheral arterial involvement in transthyretin cardiac amyloidosis cardiomyopathy

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
K Stamatelopoulos ◽  
D Delialis ◽  
D Bampatsias ◽  
M.E Tselegkidi ◽  
I Petropoulos ◽  
...  
Keyword(s):  
Risk Factors ◽  
Clinical Significance ◽  
Vascular Involvement ◽  
Control Group ◽  
Peripheral Vascular ◽  
Cvd Risk Factors ◽  
Wave Reflections ◽  
Cvd Risk ◽  
Arterial Involvement ◽  
Peripheral Arterial

Abstract Background The sporadic form of transthyretin amyloidosis cardiomyopathy (ATTR-CM) is underdiagnosed but its prevalence is increasing due to the aging population. Given the poor prognosis of ATTR-CM understanding the underlying pathophysiologic mechanisms of the disease is imperative in order to improve strategies for early diagnosis and risk stratification and to develop new effective therapeutic options. ATTR-CM is associated with hypotension and there is preliminary experimental evidence of vascular involvement but its presence and clinical significance remains unknown. Purpose To characterize peripheral arterial involvement and explore its clinical role in ATTR-CM. Methods We consecutively recruited 28 previously untreated patients with newly diagnosed ATTR-CM and 34 elderly controls &gt;70 years old, without ATTR-CM or heart failure. In both groups, flow-mediated dilatation (FMD) and intima-media thickness (IMT) in the carotid arteries were measured by high-resolution ultrasonography as markers of peripheral vascular reactivity and of subclinical atherosclerosis, respectively. Carotid-femoral pulse wave velocity (PWV) was measured as a marker of arterial stiffness. Aortic blood pressure (BP) and augmentation index (AI) using applanation tonometry were measured as markers of arterial wave reflections, peripheral arterial resistance and central hemodynamics. Echocardiography was performed in all ATTR patients. All cardiovascular (CV) measurements were performed before administration of any ATTR-specific therapy. Results ATTR patients were older and had lower prevalence of hypertension and male gender (p&lt;0.05 for all) than the control group. Aortic and peripheral BP (p=0.016–0.088) and AI (p=0.003) were lower in ATTR patients. IMT in the common (cc) and internal carotid (ic) as well as in the carotid bulb (cb) were significantly higher in ATTR patients (p=0.001–0.042). After multivariable adjustment for traditional CV disease (CVD) risk factors, the ATTR group was independently associated with AI and IMT in cc, cb and ic (p&lt;0.05 for all). In a subgroup of subjects with similar age between groups (n=13 and n=33 and 74.5±2.9 vs. 75.6±3.6 years, for ATTR vs. controls, respectively) differences in AI and cbIMT remained significant. Interestingly, AI was strongly and inversely associated with interventricular wall thickness (IVwt) in ATTR patients (spearman rho=−0.651, p=0.001). After adjustment for traditional CVD risk factors this association remained significant. Conclusion ATTR-CM is associated with lower aortic wave reflections, which correlate with more advanced structural cardiac disease, as assessed by IVwt. Further, ATTR-CM patients present accelerated subclinical carotid atherosclerosis as compared to elderly control subjects. These findings suggest that in ATTR-CM there is disease-specific peripheral vascular involvement in parallel to cardiac involvement. The clinical significance of these findings merits further investigation. Funding Acknowledgement Type of funding source: None

scholarly journals AB0608 CARDIAC VESSELS CALCIFICATION IN A COHORT OF SYSTEMIC SCLEROSIS PATIENTS: POSSIBLE ROLE IN VASCULOPATHY AND HEART ABNORMALITIES.

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1600.2-1600
Author(s):  
S. Sciacca ◽  
C. Rotondo ◽  
A. Corrado ◽  
L. Giardullo ◽  
S. Stefania ◽  
...  
Keyword(s):  
Uric Acid ◽  
Systemic Sclerosis ◽  
Disease Duration ◽  
Vascular Complications ◽  
Hypovitaminosis D ◽  
Older Age ◽  
Vascular Involvement ◽  
Cvd Risk ◽  
1 John ◽  
The Impact

Background:Cardiovascular disease is the leading cause of morbidity and mortality worldwide. Myocardial calcifications have been related with cardiovascular diseases (CVD) such as focal wall motion abnormalities and arrhythmias. The impact of vascular calcifications is under investigation in order to define the risk of cardiovascular events. The relationship between cardiac calcification and systemic sclerosis (SSc) has not been investigated.Objectives:The aim of the study is to evaluate the frequency of different patterns of cardiac calcification in SSc patients, and to correlate them to other CVD risk factors.Methods:We analyzed thoracic-CT scanners of 35 SSc patients (88% female, aged 47,8 ys ±12,9, disease duration 12,8 ys ±9) to determine the location and extension of vascular and cardiac calcification. All recruited patients fulfilled the 2013 ACR-EULAR classification criteria for SSc. No one patients had renal failure, cardiomyopathy, myocarditis, history of cardiac surgery or radiotherapy.Results:We found myocardial vessels calcifications (MCv) in 37% SSc patients, aortic wall calcifications (ACw)in 60% SSc patients, cardiac valve calcifications (VC) in 28% SSc patient and heart wall calcifications (HCw) in 20%.The SSc patients with almost one calcification had older age (65±9,8 ys vs 50±8,8 ys; p=0,0001) and higher values of circulating NTproBNP (336,9±351,9 vs 144,2±107,8; p=0,04) compared to those without.In particular, the SSc patients with MCv had and uric acid (5,3 ±1,5 vs 4,1 ±1,3; p=0,05), higher rate of PAH (25% vs 0%; p=0,037), arrhythmia (38,5% vs 9%; p=0,036) and higher prevalence of CENP-B antibodies(46% vs 4%; p=0,01) compared to patients without MCv.Patients with HCw had lower C reactive protein (0,16 ±0,10 vs 0,7±0,7; p=0,008) compared to those without HCw. No differences in the rate of heart and vascular complications of SSc were observed.The SSc patients with ACw had higher frequency of arrhythmia (33% vs 0%; p=0,016) and longer disease duration (15,5 y ±9,9 vs 8,8 ±5,8; p=0,03).The SSc patients with VC had higher rate of PAH (33%vs0%; p=0,003) and uric acid (6±0,5vs3,8±1,2 p=0,0001).Regression analysis excluded any association with gender, BMI, systemic arterial hypertension, steroid therapy, hypovitaminosis D or smoke habit. No cardiovascular event was recorded in one year of observation.Conclusion:All patterns of calcifications may be related mostly with the older age. Myocardial vessels calcifications have been found in a high percentage of SSc patients and in particular in those with PAH and positive for anti CENP-B. Furthermore, myocardial vessels calcifications could be associated to the higher occurrence of arrhythmia. More studied are needed to assess the importance of vascular calcification as a part of the vascular involvement in SSc.References:[1]John W. Nance Jr. MD. Myocardial calcifications: Pathophysiology, etiologies, differential diagnoses, and imaging findings. Journal of Cardiovascular Computed Tomography 9 (2015) 58 e 67.[2]Pagkopoulou E, Poutakidou M. Cardiovascular risk in systemic sclerosis: Micro- and Macro-vascular involvement. Indian J Rheumatol 2017;12, Suppl S1:211-7[3]Plastiras SC, Toumanidis ST. Systemic sclerosis: the heart of the matter. Hellenic J Cardiol. 2012;53(4):287–300.Disclosure of Interests:None declared

scholarly journals SAT0286 BIOLOGICAL CORRELATES OF RADIOGRAPHIC FEATURES OF INTERSTITIAL LUNG DISEASE IN SYSTEMIC SCLEROSIS: AN IN DEPTH ANALYSIS OF BRONCHOALVEOLAR PROTEINS OF SCLERODERMA LUNG STUDY I PARTICIPANTS

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1087.1-1088
Author(s):  
E. Volkmann ◽  
D. Tashkin ◽  
N. Li ◽  
G. Kim ◽  
J. Goldin ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Growth Factor ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Research Support ◽  
Radiographic Features ◽  
Depth Analysis ◽  
Specific Proteins ◽  
Clinical Measures ◽  
The Relationship

Background:Systemic sclerosis-related interstitial lung disease (SSc-ILD) involves a combination of inflammation, fibrosis and vascular pathology that is typically assessed on CT imaging as a mixture of ground-glass opacification (GGO) and fibrotic changes. We hypothesized that proteins recovered from bronchoalveolar lavage (BAL) could be used to probe the underlying pathobiology associated with GGO and fibrotic changes.Objectives:(1) To assess the relationship between 68 unique BAL proteins measured in participants of Scleroderma Lung Study (SLS) I1and radiographic and physiologic measures of ILD; (2) To identify inter-correlations among specific proteins to enlighten our understanding of how specific biological pathways contribute to SSc-ILD.Methods:Bronchoscopy was performed on 144 of the 158 participants in SLS I with 103 BAL samples available for analysis. BAL was lyophilized, concentrated 10X and used in a multiplex protein analysis for 68 different cytokines, chemokines and other factors. Kendall tau correlations were performed to assess the relationship between individual proteins and baseline measures of pulmonary function and quantitative CT scores for fibrosis, GGO and total ILD. Those proteins found to correlate significantly with at least 2 clinical measures of ILD were entered into a cluster analysis with inter-correlations expressed as a heatmap.Results:Significant correlations were observed between fibrosis scores and several biologic pathways including pro-fibrotic factors (transforming growth factor beta [TGF-β], platelet-derived growth factor [PDGF]), proteins involved in tissue remodeling (Matrix metallopeptidase [MMP]-1,7,8,9; Hepatocyte growth factor [HGF]), and those involved in monocyte/macrophage migration and activation (Monocyte chemoattractant protein [MCP]-1,3; macrophage colony-stimulating factor [MCSF]). These same pathways correlated with the diffusing capacity for carbon monoxide (DLCO). In contrast, GGO scores correlated primarily with immune and inflammatory mediators (interleukin [IL]-5,8,13,15, IL-1 receptor antagonist and interferon gamma) with only limited overlap to proteins that related to fibrosis. Vascular endothelial growth factor (VEGF) levels were lower in patients with more extensive GGO, fibrosis and diffusion impairment, suggesting that vascular changes are a central feature of SSc-ILD. Specific proteins were highly correlated with one another in a pattern suggesting biologically-related networks (Figure) that might provide additional insight regarding disease pathogenesis.Conclusion:Combining a diverse analysis of BAL proteins with the rich dataset available from SSc-ILD patients participating in SLS I, the study findings suggest the involvement of distinct biologic pathways, inter-related networks, and specific biologic signatures associated with unique radiographic features of ILD. The relationship of these factors to other SSc disease features, patient outcomes and as predictors of treatment responses will be studied in future analyses.References:[1]Tashkin DP, et al. NEJM 2006.Figure.Correlation heatmap of BAL proteins associated with at least 2 clinical measures of ILD in SSc patients. Absolute correlations are depicted, and darker colors signify stronger correlations.Disclosure of Interests:Elizabeth Volkmann Grant/research support from: Forbius, Corbus Pharmaceuticals, Consultant of: Boehringer Ingelheim, Forbius, Speakers bureau: Boehringer Ingelheim, Donald Tashkin: None declared, Ning Li: None declared, Grace Kim: None declared, Jonathan Goldin: None declared, Airi Harui: None declared, Michael Roth Grant/research support from: Genentech/Roche

Sign in / Sign up

Export Citation Format

Share Document