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In: European Journal of Echocardiography, Oxford University Press (OUP), Vol. 12, No. suppl 2 ( 2011-12-01), p. ii125-ii155

Materialart: Online-Ressource

ISSN: 1525-2167 , 1532-2114

URL: Article

DOI: 10.1093/ejechocard/jer216

Sprache: Englisch

Verlag: Oxford University Press (OUP)

Publikationsdatum: 2011

ZDB Id: 2042482-6

ZDB Id: 2647943-6

In: European Journal of Echocardiography, Oxford University Press (OUP), Vol. 11, No. Supplement 2 ( 2010-12-07), p. ii124-ii154

Materialart: Online-Ressource

ISSN: 1525-2167 , 1532-2114

URL: Article

DOI: 10.1093/ejechocard/jeq146

Sprache: Englisch

Verlag: Oxford University Press (OUP)

Publikationsdatum: 2010

ZDB Id: 2042482-6

ZDB Id: 2647943-6

In: Rheumatology, Oxford University Press (OUP), Vol. 51, No. suppl 2 ( 2012-02-01), p. ii10-ii12

Materialart: Online-Ressource

ISSN: 1462-0324 , 1462-0332

URL: Article

DOI: 10.1093/rheumatology/ker466

Sprache: Englisch

Verlag: Oxford University Press (OUP)

Publikationsdatum: 2012

ZDB Id: 1474143-X

In: Rheumatology, Oxford University Press (OUP), Vol. 51, No. suppl 2 ( 2012-02-01), p. ii17-ii19

Materialart: Online-Ressource

ISSN: 1462-0324 , 1462-0332

URL: Article

DOI: 10.1093/rheumatology/ker509

Sprache: Englisch

Verlag: Oxford University Press (OUP)

Publikationsdatum: 2012

ZDB Id: 1474143-X

In: Annals of the Rheumatic Diseases, BMJ, Vol. 80, No. Suppl 1 ( 2021-06), p. 385-386

Kurzfassung: In SSc, ILD is a major cause of morbidity and mortality. High resolution computed tomography (HRCT) is the gold standard for the diagnosis. Predictors of ILD onset are eagerly awaited to improve SSc-ILD management. Pulmonary function test (PFTs) are routinely performed to measure lung function changes. Objectives: Our aim was to investigate the performance of DLCO (diffusing capacity of lung carbon monoxide) and FVC (forced vital capacity) in predicting the development of SSc-ILD. Methods: The longitudinal data of DLCO, FVC and ILD on HRCT of SSc patients from the EUSTAR database were evaluated at baseline (t 0 ), after 12 (±4) (t 1 ) and 24 (±4) (t 2 ) months. Patients with negative HRCT for any sign of ILD both at t 0 and t 1 were included. Patients who presented or developed pulmonary hypertension during the study period were excluded. At baseline, demographic data, disease duration from Raynaud’s onset, disease subsets, autoantibodies and other laboratory and instrumental data were recorded. Results: 474/17805 patients were eligible for the study (403 females, 71 males): 26.0% dcSSc, 58.3% lcSSc, 220 (48.0%) patients with positive anticentromere antibodies (ACA) and 117 (25.4%) with positive antitopoisomerase I antibodies (Topo-I abs). Among all enrolled patients, 46 (9.7%) developed HRCT signs of ILD at t 2 . Patients with Topo-I abs showed an association with ILD development at t 2 (16.7% vs 7.8%, p=0.0031), contrarily ACA positive patients were negatively associated with ILD appearance after 2 years of follow-up (4.4% vs 14.4%, p=0.0001). Positive t 2 HRCT patients had a significant lower value of DLCO and FVC at all three assessments when compared to patients with a negative HRCT at t 2 (Table 1) and both t 0 DLCO and FVC values negatively correlated with ILD development (Table 1). The mean t 0 to t 1 change (Δ) of DLCO in patients with negative t 2 HRTC and positive t 2 HRCT were -0.5 (±12.6) and -1.0 (±15.1), respectively. The mean t 0 to t 1 ΔFVC in patients with negative t 2 HRTC and positive t 2 HRCT were -0.2 (±10.6) and 0.1 (±11.5), respectively. None of them predicted the appearance of ILD at t 2 (ΔDLCO: OR (IC) 0.997 (0.97-1.02), p=0.8024; ΔFVC OR (IC) 1.002 (0.97-1.03), p=0.8664). The data showed an association between t 0 DLCO value 〈 80% and ILD appearance after 2 years of follow-up [OR(IC): 3.09(1.49-6.40), p=0.0023]. Such association was not observed for t 0 FVC value 〈 80% [OR(IC): 1.95(0.81-4.68), p=0.1329]. The predictive capability of t 0 DLCO 〈 80% was moderate but stronger than FVC 〈 80% [AU ROC: 0.62 (0.56-0.69), 0.53 (0.48-0.59) respectively, p=0.0205]. Conclusion: Our data suggest that an impaired baseline DLCO ( 〈 80%) may have a predictive value for the development of ILD on HRCT after 2 years of follow-up. Further rigorous prospective studies are warranted to understand the role of DLCO evaluation in the course of SSc. Table 1. DLCO and FVC values at t 0 , t 1 and t 2 values in patients with positive or negative HRCT for ILD at t 2 and their statistical differences. Patients without ILD at t2 (mean±SD) Patients with ILD at t2 (mean±SD) OR (95%CL) p-value DLCO at t 0 79.0 ± 16.6 69.9 ± 17.4 0.97 (0.95 - 0.99) 0.0006 DLCO at t 1 78.4 ± 16.8 68.9 ± 18.6 0.97 (0.95 - 0.98) 0.0005 DLCO at t 2 78.0 ± 17.0 65.1 ± 19.1 0.95 (0.93 - 0.97) 〈 0.0001 FVC at t 0 102.2 ± 17.3 94.6 ± 16.2 0.97 (0.96 - 0.99) 0.0052 FVC at t 1 101.9 ± 17.9 94.7 ± 16.5 0.98 (0.96 - 0.99) 0.0092 FVC at t 2 101.6 ± 17.6 94.5 ± 20.0 0.98 (0.96 - 1) 0.0126 Disclosure of Interests: Gemma Lepri: None declared, Cosimo Bruni Speakers bureau: CB reports personal fees from Actelion, personal fees from Eli Lilly, Grant/research support from: CB reports personal fees from Actelion, personal fees from Eli Lilly, grants from European Scleroderma Trial and Research (EUSTAR) group, grants from New Horizon Fellowship, grants from Foundation for Research in Rheumatology (FOREUM), grants from Fondazione Italiana per la Ricerca sull’Artrite (FIRA), outside the submitted work, Lorenzo Tofani: None declared, Alberto Moggi Pignone: None declared, Martina Orlandi: None declared, Tomasetti Sara Speakers bureau: Speaker’s fees for Roche and Boehringer Ingelheim, Mike Hughes: None declared, Francesco Del Galdo: None declared, Rosaria Irace: None declared, Oliver Distler Grant/research support from: OD (last three years) has/had consultancy relationship and/or has received research funding in the area of potential treatments for systemic sclerosis and its complications from (last three years): Abbvie, Acceleron Pharma, Amgen, AnaMar, Arxx Therapeutics, Baecon Discovery, Blade Therapeutics, Bayer, Boehringer Ingelheim, ChemomAb, Corbus Pharmaceuticals, CSL Behring, Galapagos NV, Glenmark Pharmaceuticals, GSK, Horizon (Curzion) Pharmaceuticals, Inventiva, iQvia, Italfarmaco, iQone, Kymera Therapeutics, Lilly, Medac, Medscape, Mitsubishi Tanabe Pharma, MSD, Novartis, Pfizer, Roche, Sanofi, Serodapharm, Topadur, Target Bioscience and UCB. Patent issued “mir-29 for the treatment of systemic sclerosis” (US8247389, EP2331143)., Valeria Riccieri: None declared, Yannick Allanore Speakers bureau: YA received personal fees from Boehringer, Sanofi, Menarini and Medsenic and grants from Alpine with regards to the management of systemic sclerosis, Grant/research support from: YA received personal fees from Boehringer, Sanofi, Menarini and Medsenic and grants from Alpine with regards to the management of systemic sclerosis, Ana Maria Gheorghiu: None declared, Elise Siegert: None declared, Jeska de Vries-Bouwstra: None declared, Eric Hachulla: None declared, Mohammed Tikly: None declared, Nemanja Damjanov: None declared, Francois Spertini: None declared, Luc Mouthon: None declared, Anna-Maria Hoffmann-Vold Speakers bureau: AMHV: received consulting fees from Actelion, ARXX, Bayer, Boehringer Ingelheim, Lilly, Medscape, Merck Sharp & Dohme and Roche; and grants from Boehringer Ingelheim., Consultant of: AMHV: received consulting fees from Actelion, ARXX, Bayer, Boehringer Ingelheim, Lilly, Medscape, Merck Sharp & Dohme and Roche; and grants from Boehringer Ingelheim., Grant/research support from: AMHV: received consulting fees from Actelion, ARXX, Bayer, Boehringer Ingelheim, Lilly, Medscape, Merck Sharp & Dohme and Roche; and grants from Boehringer Ingelheim., Armando Gabrielli: None declared, Serena Guiducci: None declared, Marco Matucci-Cerinic Speakers bureau: has received consulting fees or honorarium from Actelion, Janssen, Inventiva, Bayer, Biogen, Boehringer, CSL Behring, Corbus, Galapagos, Mitsubishi, Samsung, Regeneron, Acceleron, MSD, Chemomab, Lilly, Pfizer, Roche, Grant/research support from: has received consulting fees or honorarium from Actelion, Janssen, Inventiva, Bayer, Biogen, Boehringer, CSL Behring, Corbus, Galapagos, Mitsubishi, Samsung, Regeneron, Acceleron, MSD, Chemomab, Lilly, Pfizer, Roche, Daniel Furst: None declared, Silvia Bellando Randone: None declared

Materialart: Online-Ressource

ISSN: 0003-4967 , 1468-2060

URL: Article

DOI: 10.1136/annrheumdis-2021-eular.3027

Sprache: Englisch

Verlag: BMJ

Publikationsdatum: 2021

ZDB Id: 1481557-6

In: Annals of the Rheumatic Diseases, BMJ, Vol. 80, No. Suppl 1 ( 2021-06), p. 897.2-898

Kurzfassung: COVID-19 pandemic is a global emergency which may overlap on the clinical and radiological scenario of ILD in SSc. In clinical practice, the striking similarities observed at computed tomography (CT) between the diseases make it difficult to distinguish a COVID-19 superinfection from a progression of SSc-ILD. Objectives: The aim of our study was to identify the main CT features that may help distinguishing SSc-ILD from COVID-19 pneumonia. Methods: 22 international readers were included and divided in the radiologist group (RAD) and non-radiologist group (nRAD). The RAD group included non-chest RAD and chest-RAD. A total of 99 patients, 52 with COVID-19 and 47 with SSc-ILD, were included in the study. Results: Fibrosis inside focal ground glass opacities (GGO) in the upper lobes; fibrosis in the lower lobe GGO; reticulations in lower lobes (especially if bilateral and symmetrical or associated with signs of fibrosis) were the CT parameters most frequently associated with SSc-ILD. The CT parameters most frequently associated with COVID- 19 pneumonia were: consolidation (CONS) in the lower lobes, CONS with peripheral (both central/peripheral or patchy distributions), anterior and posterior CONS and rounded-shaped GGOs in the lower lobes. After multivariate analysis, the presence of CONS in the lower lobes (p 〈 0.0001) and signs of fibrosis in GGO in the lower lobes (p 〈 0.0001) remained independently associated with COVID-19 pneumonia or SSc-ILD, respectively. These two variables were combined in a predictive score which resulted positively associated with the COVID-19 diagnosis, with 96.1% sensitivity and 83.3% specificity: 3 different risk class for COVID-19 pneumonia may be identified: high risk for COVID-19 pneumonia (5-9 points); probable overlap COVID-19 pneumonia in SSc-ILD (4 points); low risk for COVID-19 pneumonia (0-3 points). Conclusion: The CT differential diagnosis between COVID-19 Pneumonia and SSc-ILD is possible and may be fostered in practice by the use of a radiological score. In the case where an overlap of both diseases is suspected, the presence of consolidation in the lower lobes may suggest a COVID-19 pneumonia while the presence of fibrosis inside GGO may indicate a SSc-ILD. References: [1]Orlandi M, Landini N, Bruni C, et al. Infection or autoimmunity? The clinical challenge of interstitial lung disease in systemic sclerosis during COVID 19 pandemic. J Rheumatol. 2020 Dec 1: jrheum.200832 [2]Simpson S, Kay FU, Abbara S, et al. Radiological Society of North America Expert Consensus Statement on Reporting Chest CT Findings Related to COVID-19. Endorsed by the Society of Thoracic Radiology, the American College of Radiology, and RSNA [published online ahead of print, 2020 Apr 28] . J Thorac Imaging. 2020;10.1097/RTI.0000000000000524. [3]Cheng C, Li C, Zhao T, et al. COVID-19 with rheumatic diseases: a report of 5 cases. Clin Rheumatol. 2020;39(7):2025-2029. [4]Mariano RZ, Rio APTD, Reis F. Covid-19 overlapping with systemic sclerosis. Rev Soc Bras Med Trop. 2020 Sep 21;53:e20200450. Disclosure of Interests: None declared

Materialart: Online-Ressource

ISSN: 0003-4967 , 1468-2060

URL: Article

DOI: 10.1136/annrheumdis-2021-eular.2967

Sprache: Englisch

Verlag: BMJ

Publikationsdatum: 2021

ZDB Id: 1481557-6

In: European Heart Journal, Oxford University Press (OUP), Vol. 41, No. Supplement_2 ( 2020-11-01)

Kurzfassung: Malignant arrhythmias due to cardiac involvement are a frequent cause of death in systemic sclerosis (SSc). Cardiac involvement, which is linked to both ischaemic and non-ischaemic fibrotic deposition, is often subclinical. Cardiovascular magnetic resonance (CMR) is the non-invasive gold standard for myocardial tissue characterization and can detect macroscopic myocardial fibrosis through late Gadolinium enhancement (LGE). Aim To evaluate the role of LGE to predict malignant arrhythmias requiring implantable cardioverter defibrillator (ICD) in SSc. Methods 289 SSc patients underwent a thorough clinical evaluation and CMR exam using a 1.5 T scanner. Biventricular function parameter by SSFP cine images, oedema by STIR T2 images, and macroscopic fibrosis by LGE were assessed. Patients were followed-up and malignant ventricular arrhythmias requiring ICD implantation was considered as event. Results Out of 289 patients, 111 (38.4%) showed LGE and 83/111 (28.7% of the total population) showed a non-junctional distribution. During the follow-up (45±27 months), 10 patients needed ICD after malignant ventricular arrhythmias (7 patients with LGE, 3 patients without LGE). CMR predictors of cardiac events by univariate analysis were left and right ventricular ejection fractions, indexed right atrial area and non-junctional LGE. At multivariate analysis, macroscopic myocardial fibrosis detected by LGE was an independent predictor (hazard ratio 5.4; 95% C.I. 1.1–28.8, p & lt;0.05; see figure for Kaplan-Meier curves). Conclusions Presence of ventricular LGE at CMR may represent an independent predictor for further malignant ventricular arrhythmias requiring ICD implantation in SSc patients. Kaplan-Meier curves Funding Acknowledgement Type of funding source: None

Materialart: Online-Ressource

ISSN: 0195-668X , 1522-9645

URL: Article

DOI: 10.1093/ehjci/ehaa946.0219

Sprache: Englisch

Verlag: Oxford University Press (OUP)

Publikationsdatum: 2020

ZDB Id: 2001908-7

In: Annals of the Rheumatic Diseases, BMJ, Vol. 80, No. Suppl 1 ( 2021-06), p. 105.2-106

Kurzfassung: Gastrointestinal involvement is one of the most frequent features of SSc, affecting nearly 90% of patients, with a great impact on quality of life and morbidity. One of the key pathological factors of SSc bowel involvement is vasculopathy (1), although little is known about its pathophysiology and no treatments are currently available. Objectives: to assess with abdominal US the superior mesenteric artery (SMA) and the inferior mesenteric artery (IMA) vessel characteristics and blood flow in SSc, compared to healthy controls (HC). Methods: we performed fasting abdominal US in SSc patients fulfilling the ACR/EULAR 2013 classification criteria and HC. Patients with a history of peripheral /coronary arterial disease were excluded. For both SMA and IMA, caliber (mm), Peak Systolic Velocity – PSV (cm/sec), Reverse Velocity – RV (cm/sec), End-Diastolic Velocity – EDV (cm/sec), Mean Velocity – mV (cm/sec), Blood-flow (cm/sec), Resistive Index – RI and Pulsatility Index – PI were measured. Results: 28 SSc patients [25 females (89.3%), mean age 48.75 ± 12.39 years; 6 (22.22%) anti-centromere and 19 (70.37%) positive for anti-topoisomerase I antibodies] and 28 HC [18 females (64.3%), mean age 36.25 ± 12.08 years] were evaluated. In SSc, the SMA caliber was significantly smaller than in HC (5.75 ± 0.62 vs. 6.45 ± 0.60 mm, p 〈 0.0001), while IMA dimensions did not differ. The SMA study revealed SSc patients had a significant reduction of RV (7.25 ± 6.37 vs. 18.52 ± 6.16 cm/sec, p 〈 0.0001) and PI (3.33 ± 0.75 vs. 4.53 ± 1.03, p 〈 0.0001) when compared to HC. In addition, in SSc the mV of SMA was significantly lower than in HC (38.03 ± 13.90 vs. 28.32 ±9.25 cm/sec, p=0.0035), as well as the RI (0.88 ± 0.04 vs. 0.91 ± 0.03, p=0.0034); EDV was significantly increased (16.34 ± 7.03 vs. 12.64 ± 5.46 cm/sec, p=0.0321). Similarly to SMA, also in IMA RV and PI were significantly lower that controls (RV: 2.69 ± 6.10 vs. 17.06 ± 5.75 cm/sec, p 〈 0.0001; PI: 3.54 ± 0.95 vs. 6.08 ± 1.53, p 〈 0.0001). Moreover SSc patients presented a significant reduction of PSV and RI of IMA (PSV: 72.27 ± 27.23 vs. 93.81 ± 25.73 cm/sec, p=0.0084; RI: 0.88 ± 0.04 vs. 0.91 ± 0.03, p=0.0132) when compared to HC. Although the HC group was significantly younger than the SSc group (p=0.0003), all the results were confirmed after adjustment for age (Table 1). Table 1. comparison of the characteristics of SMA and IMA between SSc patients and HC. SSc HC p-value Age adjusted p-value SMA N Mean ± SD N Mean ± SD Caliber (mm) 28 5.75 ± 0.62 28 6.45 ± 0.60 〈 0.0001 0.0002 PSV (cm/sec) 28 137.50 ± 34.50 28 135.26 ± 33.81 0.8075 0.7297 RV (cm/sec) 28 7.25 ± 6.37 28 18.52 ± 6.16 〈 0.0001 〈 0.0001 EDV (cm/sec) 28 16.34 ± 7.03 28 12.64 ± 5.46 0.0321 0.0650 mV (cm/sec) 28 38.03 ± 13.90 28 28.32 ±9.25 0.0035 0.0150 Blood-flow (cm/sec) 28 1073.1 ± 831.16 28 913.36 ± 272.87 0.3409 0.4781 PI 28 3.33 ± 0.75 28 4.53 ± 1.03 〈 0.0001 0.0002 RI 28 0.88 ± 0.04 28 0.91 ± 0.03 0.0034 0.0141 IMA Caliber (mm) 26 2.71 ± 0.47 24 2.79 ± 0.37 0.4872 0.5385 PSV (cm/sec) 23 72.27 ± 27.23 23 93.81 ± 25.73 0.0084 0.0044 RV (cm/sec) 23 2.69 ± 6.10 23 17.06 ± 5.75 〈 0.0001 〈 0.0001 EDV (cm/sec) 23 7.87 ± 2.01 23 7.95 ± 2.10 0.8921 0.9250 mV (cm/sec) 23 17.83 ± 5.33 23 14.75 ± 5.08 0.0514 0.3938 Blood-flow (cm/sec) 23 106.70 ± 47.99 20 84.00 ± 30.13 0.0676 0.3056 PI 23 3.54 ± 0.95 23 6.08 ± 1.53 〈 0.0001 〈 0.0001 RI 23 0.88 ± 0.04 23 0.91 ± 0.03 0.0132 0.0205 SMA=superior mesentheric artery, IMA=inferior mesentheric artery, PSV=Peak Systolic Velocity, RV=Reverse Velocity, EDV=End-Diastolic Velocity, mV=Mean Velocity, PI=Pulsatility Index, RI=Resistive Index. Conclusion: this preliminary study shows, for the first time, the presence of a significant reduction of RV, PI and RI in the intestinal arteries of SSc patients when compared to HC. These data show an increased stiffness of the gastrointestinal arterial wall, in agreement with the typical SSc vasculopathy. A larger cohort is needed to confirm the results and explore the possible relationship with other clinical features of the disease. References: [1] Sjogren, RW. Gastrointestinal features of scleroderma. Curr Opin Rheumatol 1996;8:569-75. Disclosure of Interests: None declared

Materialart: Online-Ressource

ISSN: 0003-4967 , 1468-2060

URL: Article

DOI: 10.1136/annrheumdis-2021-eular.647

Sprache: Englisch

Verlag: BMJ

Publikationsdatum: 2021

ZDB Id: 1481557-6

In: European Heart Journal, Oxford University Press (OUP), Vol. 40, No. Supplement_1 ( 2019-10-01)

Kurzfassung: Patients with systemic sclerosis (SSc) are at risk of developing pulmonary arterial hypertension (PAH), which is often diagnosed late when the benefits of vasoactive therapies are limited. The concept of exercise pulmonary hypertension as a possible transitional phase anticipating resting PAH has been assessed in several pathologies, but has not been endorsed by the latest European Guidelines, because not supported by sufficient data. Purpose To evaluate whether PASP values at rest and at peak exercise, estimated at echocardiography, could be predictors of further development of PAH. Methods Four hundred and twenty-nine SSc patients without a previous diagnosis of PAH, enrolled at 4 referral Centres, underwent standard exercise Doppler echocardiography with PASP estimation at rest and at peak stress. Patients were then followed-up to assess the development of PAH, as diagnosed by a complete diagnostic work-up including right heart catheterization. PAH was defined by pre-capillary pulmonary hypertension (mean pulmonary artery pressure ≥25 mmHg with pulmonary arterial wedge pressure ≤15 mmHg), without significant interstitial lung disease and/or left heart disease. Results During the median follow-up of 75 months (IQR 29–114), 16 patients developed PAH. A combined cut-off of ≥24 mmHg as resting PASP and ≥40 as peak PASP was identified as the best predictor of further development of PAH (see Figure). Both resting PASP and peak PASP were predictors of PAH at univariate analysis (resting PASP OR 1.13, 95% C.I. 1.07–1.19, p 〈 0.0001; peak PASP OR 1.13, 95% C.I. 1.07–1.18, p 〈 0.0001). At multivariate analysis, only peak PASP was independently associated to PAH development (OR 1.13, 95% C.I. 1.04–1.18, p 〈 0.001). Only one patient among those with resting PASP 〈 24 mmHg and peak PASP 〈 40 mmHg (34.7% of the total population) developed PAH during the follow-up (after 10 years from normal exercise Doppler echocardiography). Kaplan-Meier curves Conclusions Exercise increase in PASP is an independent predictor of later development of PAH in SSc. An increase in exercise PASP is frequent and is not necessarily associated with a later development of PAH, whereas the very high negative predictive value of a normal PASP both at rest and at peak exercise can be used in the clinical practice to confidently rule out about one third of patients. Acknowledgement/Funding Italian Ministry of Health (Ricerca Finalizzata 2011-2012)

Materialart: Online-Ressource

ISSN: 0195-668X , 1522-9645

URL: Article

DOI: 10.1093/eurheartj/ehz746.1069

Sprache: Englisch

Verlag: Oxford University Press (OUP)

Publikationsdatum: 2019

ZDB Id: 2001908-7

In: Annals of the Rheumatic Diseases, BMJ, Vol. 79, No. Suppl 1 ( 2020-06), p. 409.2-409

Kurzfassung: Cardiac involvement in systemic sclerosis (SSc) accounts for 26-36% of deaths. This most frequently manifests as ventricular rhythm disturbances (VRDs), eventually culminating in sudden cardiac death. However, no specific guidelines exist for implantation of cardioverter defibrillators (ICD) in SSc patients. Parametric cardiovascular magnetic resonance (CMR) indices of myocardial oedema and fibrosis like native T1/T2 mapping have been shown to be associated with prognosis in SSc patients with acute cardiac events and normal echocardiograms. However, their relationship with arrhythmogenicity per se has not been previously investigated in SSc. Objectives: To investigate the relationship between parametric CMR indices and arrhythmogenicity in SSc patients. Methods: 84 consecutive SSc patients (80% diffuse-cutaneous SSc) from eight European centers presenting with cardiac symptoms were examined using a 1.5 T CMR system. 24h Holter recordings were obtained within a month of the CMR scan. The presence of VRDs was defined as any type of premature ventricular contraction (PVC) in couples, triplets, bigeminism, trigeminism, quadrigeminism and non-sustained ventricular tachycardia, as well as having 〉 30 PVCs per hour. Logistic regression analysis was used to evaluate the relationship between VRD occurrence and native T1/T2 mapping as well as myocardial extracellular volume fraction (ECV). Results: Mean age in the cohort was 55 (13) years and 78 (93%) patients were female. Of these, 67 (80%) experienced at least one type of VRDs. Each 10 ms increase of native T1-mapping was associated with a higher occurrence of VRDs [odds ratio (95% confidence interval): 1.21 (1.08-1.36), p=0.001]. Similarly, a 1% increase in ECV conferred an increased probability of experiencing VRDs [1.25 (1.01-1.53), p=0.037] . Lastly, a 1ms unit increase in T2-mapping also led to increased probability of having experienced VRDs [1.09 (1.01-1.19), p=0.035]. Conclusion: Parametric CMR indices are associated with arrhythmogenicity in SSc patients with cardiac symptoms and should be investigated further in larger studies for their clinical utility in selecting high-risk SSc patients for ICD implantation. Disclosure of Interests: Sophie I. Mavrogeni: None declared, Luna Gargani: None declared, Alessia Pepe: None declared, Lorenzo Monti: None declared, George Markousis-Mavrogenis: None declared, Maria De Santis: None declared, Antonella Meloni: None declared, Loukia Koutsogeorgopoulou: None declared, Georgia Karabela: None declared, Efthymios Stavropoulos: None declared, Gkikas Katsifis Grant/research support from: UCB Pharma, Janssen, Abbvie, Novartis, MSD, Aenorasis, Genesis Pharma, Pfizer, Roche, Consultant of: UCB Pharma, Janssen, Abbvie, Novartis, MSD, Aenorasis, Genesis Pharma, Pfizer, Roche, Speakers bureau: UCB Pharma, Janssen, Abbvie, Novartis, MSD, Aenorasis, Genesis Pharma, Pfizer, Roche, Konstantinos Bratis: None declared, Silvia Bellando Randone: None declared, Serena Guiducci: None declared, Cosimo Bruni: None declared, Alberto Moggi-Pignone: None declared, Theodoros Dimitroulas: None declared, Paraskevi Voulgari: None declared, Genovefa Kolovou: None declared, Vasiliki-Kalliopi Bournia Grant/research support from: Travel Grant from Boehringer Ingelheim, Monica Mukherjee: None declared, Joao Lima: None declared, George D. Kitas: None declared, Petros Sfikakis Grant/research support from: Grant/research support from Abvie, Novartis, MSD, Actelion, Amgen, Pfizer, Janssen Pharmaceutical, UCB, Marco Matucci-Cerinic Grant/research support from: Actelion, MSD, Bristol-Myers Squibb, Speakers bureau: Acetelion, Lilly, Boehringer Ingelheim

Materialart: Online-Ressource

ISSN: 0003-4967 , 1468-2060

URL: Article

DOI: 10.1136/annrheumdis-2020-eular.6575

Sprache: Englisch

Verlag: BMJ

Publikationsdatum: 2020

ZDB Id: 1481557-6