scholarly journals THU0351 MUSCLE INVOLVEMENT IN PATIENTS WITH SYSTEMIC SCLEROSIS AND ANTI-PM/SCL+ ANTIBODIES IS ASSOCIATED WITH CARDIAC AND PULMONARY INVOLVEMENT. ANALYSIS OF THE MULTICENTRE EUSTAR COHORT.

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 407.1-407
Author(s):  
M. G. Lazzaroni ◽  
S. Zingarelli ◽  
P. Airò ◽  
Y. Allanore ◽  
O. Distler
Keyword(s):  
Systemic Sclerosis ◽  
Cardiac Involvement ◽  
Severe Disease ◽  
Disease Onset ◽  
Pulmonary Involvement ◽  
Left Ventricular ◽  
Research Support ◽  
Muscle Involvement ◽  
Group A ◽  
Tendon Friction Rubs

Background:Anti-PM/Scl antibodies positivity has been associated with frequent skeletal muscle involvement in patients with Systemic Sclerosis (SSc) in different studies, including the EUSTAR cohort (1). Moreover, although myositis has been previously associated with heart involvement in SSc patients (2), this issue has never been explored among anti-PM/Scl+ patients.Objectives:To evaluate the cardiac involvement in anti-PM/Scl patients with SSc in the large multicentre EUSTAR database, with focus on the subgroup of patients with muscle involvement.Methods:Patients from the EUSTAR database were included when the item anti-PM/Scl was fulfilled in at least one visit.Results:Anti-PM/Scl status was available in 7,353 SSc patients from EUSTAR database: 295 were anti-PM/Scl+. After exclusion of 151 patients with multiple autoantibody positivity, 144 anti-PM/Scl + patients were compared with 7,058 anti-PM/Scl- patients. Among them, 3,120 (44.2%) were positive for ACA, 2,361 (33.5%) for anti-Topo I and 274 (3.88%) for anti-RNAP3.Regarding the specific cardiac outcomes, in the anti-PM/Scl+ as compared to the anti-PM/Scl- group, a decreased rate of elevated sPAP at ECHO was recorded (12.8% vs 25.0%, p:0.001), while no differences were observed in the frequency of conduction blocks (26.2% vs 23.7%, p:0.526), abnormal diastolic function (33.9% vs 36.4%, p:0.582), pericardial effusion (10.2% vs 10.9%, p:1.000) and LVEF ≤50% (4.76% vs 6.11%, p:0.818). In multivariate analysis, adjusted for age at disease onset, sex, and disease duration, the negative association of anti-PM/Scl with elevated sPAP was not confirmed (p:0.061).When comparing anti-PM/Scl+ patients with (n=47) and without (n=87) CK elevation, the former group had a higher frequency of conduction blocks (43.2% vs 17.5%, p:0.005; OR 95% CI 3.47, 1.51-7.97) and left ventricular dysfunction, both diastolic (45.6% vs 27.2%, p:0.050; OR 95% CI 2.25, 1.05-4.81) and systolic (LVEF ≤50% 13.3% vs 0%, p:0.018; OR 95% CI 16.8, 0.87-324). Moreover, anti-PM/Scl+ patients with CK elevation had significantly increased rate of lung fibrosis on HRCT (p:0.045), intestinal symptoms (p:0.017), joint contractures (p:0.045) and tendon friction rubs (p:0.034).Conclusion:In the largest series of anti-PM/Scl positive SSc patients so far reported, muscle involvement in anti-PM/Scl+ patients (defined as increased serum CK) seems to represent a marker of a more severe disease phenotype, including a higher frequency of cardio-pulmonary involvement.References:[1]Lazzaroni MG, et al. Ann Rheum Dis 2018. 77 (2), 421-2.[2]Follansbee WP, et al. Am Heart J 1993. 125: 194-203.Acknowledgments:Authors would like to thank the patients’ association GILS (Gruppo Italiano Lotta Sclerodermia) for the grant that supported the project.Disclosure of Interests:Maria Grazia Lazzaroni: None declared, Stefania Zingarelli: None declared, Paolo Airò: None declared, Yannick Allanore Grant/research support from: BMS, Inventiva, Roche, Sanofi, Consultant of: Actelion, Bayer AG, BMS, BI, Oliver Distler Grant/research support from: Grants/Research support from Actelion, Bayer, Boehringer Ingelheim, Competitive Drug Development International Ltd. and Mitsubishi Tanabe; he also holds the issued Patent on mir-29 for the treatment of systemic sclerosis (US8247389, EP2331143)., Consultant of: Consultancy fees from Actelion, Acceleron Pharma, AnaMar, Bayer, Baecon Discovery, Blade Therapeutics, Boehringer, CSL Behring, Catenion, ChemomAb, Curzion Pharmaceuticals, Ergonex, Galapagos NV, GSK, Glenmark Pharmaceuticals, Inventiva, Italfarmaco, iQvia, medac, Medscape, Mitsubishi Tanabe Pharma, MSD, Roche, Sanofi and UCB, Speakers bureau: Speaker fees from Actelion, Bayer, Boehringer Ingelheim, Medscape, Pfizer and Roche

THU0499 IS THERE A DIFFERENT PRESENTATION OF JUVENILE SYSTEMIC DIFFUSE AND LIMITED SUBSET? DATA FROM THE JUVENILE SCLERODERMA INCEPTION COHORT. WWW.JUVENILE-SCLEORDERMA.COM

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 487-488
Author(s):  
I. Foeldvari ◽  
J. Klotsche ◽  
O. Kasapcopur ◽  
A. Adrovic ◽  
K. Torok ◽  
...  
Keyword(s):  
Global Assessment ◽  
Cardiac Involvement ◽  
Severe Disease ◽  
Pulmonary Involvement ◽  
Systemic Scleroderma ◽  
Systemic Hypertension ◽  
Physician Global Assessment ◽  
Inception Cohort ◽  
Research Support ◽  
The Mean

Background:Juvenile systemic scleroderma (jSSc) is an orphan disease with a prevalence of 3 per 1 000 000 children. There are limited data regarding the clinical presentation of jSSc. The Juvenile Systemic Scleroderma Inception Cohort (JSSIC) is the largest multinational registry that prospectively collects information about jSSc patients.Objectives:Evaluation of the jSSc patients at the time of inclusion in the JSSIC.Methods:Patients were included in the JSSIC if they fulfilled the adult ACR/EULAR classification criteria for systemic scleroderma, if they presented the first non-Raynaud symptom before 16 years of age and if they were younger than 18 years of age at time of inclusion. Patients’ characteristics at time of inclusion were evaluated.Results:Until 15thof December 2019 hundred fifty patients were included, 83% of them being Caucasian and 80% female. The majority had the diffuse subtype (72%) and 17% of all jSSc had overlap features. The mean age of first presentation of Raynaud´s phenomenon was 9.8 years in the diffuse subtype (djSSc) and 10.7 years in the limited subtype (ljSSc) (p=.197). The mean age at first non-Raynaud’s symptoms was 10.0 years in the djSSc and 11.2 years in the ljSSc (p=0.247). Mean disease duration at time of inclusion was 3.4 years in the djSSc and 2.4 years in the ljSSc group.Significant differences were found between the groups regarding mean modified Rodnan skin score, 18.2 in the djSSc vs 6.2 in the ljSSc (p=0.02); presence of Gottron´s papulae (djSSc 30% vs ljSSc 13%, p=0.43);presence of teleangiectasia (djSSc 42% vs 18% ljSS, p=0.01); history of ulceration (djSSc 42% vs 18% ljSSc,p=0.008); 6 Minute walk test below the 10thpercentile (djSSc 85% vs ljSSc 54%, p=0.044), total pulmonary involvement (djSSc 49% vs ljSSc 31%, p=0.045), cardiac involvement (ljSSc 17% vs djSSc 3%, p=0.002). djSSc patients had significantly worse scores for Physician Global Assessment of disease activity compared to ljSSc patients (VAS 0-100) (40 vs 15) (p=0.001) and for Physician Global Assessment of disease damage (VAS 0-100) (36 vs 17) (p=0.001).There were no statistically significant differences in the other presentations. Pulmonary hypertension occurred in approximately 6% in both groups. No systemic hypertension or renal crisis was reported. ANA positivity was 90% in both groups. Anti-Scl70 was positive in 35% in djSSc and 36% in the ljSSc group. Anticentromere positivity occurred in 3% in the djSSc and 7% in the ljSSc group.Conclusion:In this unique large cohort of jSSc patients there were significant differences between djSSc and ljSSc patients at time of inclusion into the cohort regarding skin, vascular, pulmonary and cardiac involvement. According to the physician global scores the djSSc patients had a significantly more severe disease. Interestingly the antibody profile was similar in both scleroderma phenotypes.Supported by the “Joachim Herz Stiftung”Disclosure of Interests: :Ivan Foeldvari Consultant of: Novartis, Jens Klotsche: None declared, Ozgur Kasapcopur: None declared, Amra Adrovic: None declared, Kathryn Torok: None declared, Maria T. Terreri: None declared, Ana Paula Sakamoto: None declared, Valda Stanevicha: None declared, Flávio R. Sztajnbok: None declared, Jordi Anton Grant/research support from: grants from Pfizer, abbvie, Novartis, Sobi. Gebro, Roche, Novimmune, Sanofi, Lilly, Amgen, Grant/research support from: Pfizer, abbvie, Novartis, Sobi. Gebro, Roche, Novimmune, Sanofi, Lilly, Amgen, Consultant of: Novartis, Sobi, Pfizer, abbvie, Consultant of: Novartis, Sobi, Pfizer, abbvie, Speakers bureau: abbvie, Pfizer, Roche, Novartis, Sobi, Gebro, Speakers bureau: abbvie, Pfizer, Roche, Novartis, Sobi, Gebro, Brian Feldman Consultant of: DSMB for Pfizer, OPTUM and AB2-Bio, Ekaterina Alexeeva Grant/research support from: Roche, Pfizer, Centocor, Novartis, Speakers bureau: Roche, Novartis, Pfizer., Maria Katsikas: None declared, Vanessa Smith Grant/research support from: The affiliated company received grants from Research Foundation - Flanders (FWO), Belgian Fund for Scientific Research in Rheumatic diseases (FWRO), Boehringer Ingelheim Pharma GmbH & Co and Janssen-Cilag NV, Consultant of: Boehringer-Ingelheim Pharma GmbH & Co, Speakers bureau: Actelion Pharmaceuticals Ltd, Boehringer-Ingelheim Pharma GmbH & Co and UCB Biopharma Sprl, edoardo marrani: None declared, Mikhail Kostik: None declared, Natalia Vasquez-Canizares: None declared, Simone Appenzeller: None declared, Mahesh Janarthanan: None declared, Monika Moll: None declared, Dana Nemcova: None declared, Anjali Patwardhan: None declared, Maria Jose Santos Speakers bureau: Novartis and Pfizer, Sujata Sawhney: None declared, Dieneke Schonenberg: None declared, Cristina Battagliotti: None declared, Lillemor Berntson Consultant of: paid by Abbvie as a consultant, Speakers bureau: paid by Abbvie for giving speaches about JIA, Blanca Bica: None declared, Juergen Brunner Grant/research support from: Pfizer, Novartis, Consultant of: Pfizer, Novartis, Abbvie, Roche, BMS, Speakers bureau: Pfizer, Novartis, Abbvie, Roche, BMS, Patricia Costa Reis: None declared, Despina Eleftheriou: None declared, Liora Harel: None declared, Gerd Horneff Grant/research support from: AbbVie, Chugai, Merck Sharp & Dohme, Novartis, Pfizer, Roche, Speakers bureau: AbbVie, Bayer, Chugai, Merck Sharp & Dohme, Novartis, Pfizer, Roche, Daniela Kaiser: None declared, Dragana Lazarevic: None declared, Kirsten Minden Consultant of: GlaxoSmithKline, Sanofi, Speakers bureau: Roche, Susan Nielsen: None declared, Farzana Nuruzzaman: None declared, Yosef Uziel: None declared, Nicola Helmus: None declared

Rituximab for rapidly progressive juvenile systemic sclerosis

Rheumatology ◽  
2020 ◽  
Vol 59 (12) ◽  
pp. 3793-3797 ◽  
Author(s):  
Francesco Zulian ◽  
Roberto Dal Pozzolo ◽  
Alessandra Meneghel ◽  
Biagio Castaldi ◽  
Renzo Marcolongo ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Cardiac Involvement ◽  
Myocardial Function ◽  
Oral Prednisone ◽  
Pulmonary Involvement ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Rapid Progression ◽  
Ventricular Ejection Fraction ◽  
Juvenile Systemic Sclerosis

Abstract Objective Juvenile systemic sclerosis (JSSc) with rapidly progressive course is a life-threatening condition associated with a poor prognosis. Recently, rituximab (RTX) has been shown to be a promising treatment for adult patients with SSc. We present a series of four patients with rapidly progressive JSSc successfully treated with RTX. Methods Clinical, laboratory and functional parameters were collected from four patients with rapidly progressive JSSc treated with RTX for at least 1 year. All patients underwent four yearly courses of i.v. RTX 375 mg/m2 on day 0 and 14, at 3-month intervals. Low dose oral prednisone and MMF were also administered. Data were recorded at baseline and every 6 months and included pulmonary and myocardial function parameters, muscular, vascular and skin changes. The Juvenile Systemic Sclerosis Severity Score (J4S) estimated the overall disease severity over time. Results Four patients (three males, one female), aged 8–17 years, entered the study. Three patients presented with prevalent cardiac involvement, one with severe pulmonary involvement. After 1 year of RTX treatment, all patients showed significant improvement of J4S, Raynaud’s phenomenon and cutaneous involvement. Among those with prevalent cardiac involvement, two showed an improvement of the myocardial function (left ventricular ejection fraction [EF] +37% and +19%, respectively) and in the third arrhythmias disappeared. The patient with severe pulmonary involvement showed a significant improvement of the respiratory function (forced vital capacity +46%, forced expiratory volume in 1 s +33%, diffusing capacity of the lung for carbon monoxide [DLCO] +30%). No major side effects were reported. Conclusions Our data suggest that a combination of RTX and MMF is effective in arresting the rapid progression of JSSc.

scholarly journals Epidemiology of systemic sclerosis: a multi-database population-based study in Tuscany (Italy)

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Alessio Coi ◽  
◽  
Simone Barsotti ◽  
Michele Santoro ◽  
Fabio Almerigogna ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Rare Diseases ◽  
Severe Disease ◽  
Pulmonary Involvement ◽  
Population Based ◽  
Vital Statistics ◽  
Drug Prescriptions ◽  
Malignant Neoplasms ◽  
Lower Survival ◽  
Treatment And Prevention

Abstract Background Systemic Sclerosis (SSc) is a chronic autoimmune disease with a complex pathogenesis that includes vascular injury, abnormal immune activation, and tissue fibrosis. We provided a complete epidemiological characterization of SSc in the Tuscany region (Italy), considering prevalence and incidence, survival, comorbidities and drug prescriptions, by using a multi-database population-based approach. Cases of SSc diagnosed between 1st January 2003 and 31st December 2017 among residents in Tuscany were collected from the population-based Rare Diseases Registry of Tuscany. All cases were linked to regional health and demographic databases to obtain information about vital statistics, principal causes of hospitalization, complications and comorbidities, and drug prescriptions. Results The prevalence of SSc in Tuscany population resulted to be 22.2 per 100,000, with the highest prevalence observed for the cases aged ≥ 65 years (33.2 per 100,000, CI 95% 29.6–37.3). In females, SSc was predominant (86.7% on the total) with an overall sex ratio F/M of 6.5. Nevertheless, males presented a more severe disease, with a lower survival and significant differences in respiratory complications and metabolic comorbidities. Complications and comorbidities such as pulmonary involvement (HR = 1.66, CI 95% 1.17–2.35), congestive heart failure (HR = 2.76, CI 95% 1.80–4.25), subarachnoid and intracerebral haemorrhage (HR = 2.33, CI 95% 1.21–4.48) and malignant neoplasms (HR = 1.63, CI 95% 1.06–2.52), were significantly associated to a lower survival, also after adjustment for age, sex and other SSc-related complications. Disease-modifying antirheumatic drugs, endothelin receptor antagonists, and phosphodiesterase-5 inhibitors were the drugs with the more increasing prevalence of use in the 2008–2017 period. Conclusions The multi-database approach is important in the investigation of rare diseases where it is often difficult to provide accurate epidemiological indicators. A population-based registry can be exploited in synergy with health databases, to provide evidence related to disease outcomes and therapies and to assess the burden of disease, relying on a large cohort of cases. Building an integrated archive of data from multiple databases linking a cohort of patients to their comorbidities, clinical outcomes and survival, is important both in terms of treatment and prevention.

scholarly journals SAT0500 HOW THE ADULT CRISS WORKS IN PEDIATRIC jSSc PATIENTS - RESULTS FROM THE JUVENILE SCLERODERMA INCEPTION COHORT

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1206.2-1206
Author(s):  
J. Klotsche ◽  
I. Foeldvari ◽  
O. Kasapcopur ◽  
A. Adrovic ◽  
K. Torok ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Area Under Curve ◽  
Health Assessment ◽  
Organ Damage ◽  
Left Ventricular ◽  
Inception Cohort ◽  
Research Support ◽  
Predicted Probability ◽  
New Onset

Background:The Composite Response Index in Systemic Sclerosis (CRISS) was developed by Dinesh Khanna as a response measure in patients with adult systemic sclerosis. CRISS aims to capture the complexity of systemic sclerosis and to provide a sensitive measure for change in disease activity. The CRISS score is based on a two-step approach. First, significant disease worsening or new-onset organ damage is defined as non-responsiveness. In patients who did not fulfill the criteria of part one, a probability of improvement is calculated for each patient based the Rodnan Skin Score (mRSS), percent predicted forced vital capacity (FVC%), patient and physician global assessments (PGA), and the Health Assessment Questionnaire Disability Index (HAQ-DI). A probability of 0.6 or higher indicates improvement.Objectives:The objective of this study was to validate the CRISS in a prospectively followed cohort of patients with juvenile systemic sclerosis (jSSc).Methods:Data from the prospective international inception cohort for jSSc was used to validate the CRISS. Patients with an available 12-months follow-up were included in the analyses. Clinically improvement was defined by the anchor question about improvement (much better or little better versus almost the same, little worse or much worse) in patients overall health due to scleroderma since the last visit provided by the treating physician.Results:Forty seven jSSc patients were included in the analysis. 74.2% had diffuse subtype. The physician rated the disease as improved in 34 patients (72.3%) since the last visit. No patient had a renal crisis or new onset of left ventricular failure during the 12-months follow-up. Three patients (3.4%) each had a new onset or worsening of lung fibrosis and new onset of pulmonary arterial hypertension. In total, 6 patients resulted in a rating of not improved based on the CRISS in part I. The mRSSS, FVC%, CHAQ and PGA significantly improved during the 12-months follow-up in patients who were rated as improved. The predicted probability based on the CRISS algorithm resulted in an area under curve of 0.77 predicting the anchor question of improvement. In summary, 33 (70.0%) patients were correctly classified by the adult CRISS score resulting in an overall area under curve of 0.7.Conclusion:The CRISS score was evaluated in a pediatric jSSc cohort for the first time. It showed a good performance. However, it seems that the formula of part II of the CRISS score needs a calibration to pediatric jSSc patients.Disclosure of Interests:Jens Klotsche: None declared, Ivan Foeldvari Consultant of: Novartis, Ozgur Kasapcopur: None declared, Amra Adrovic: None declared, Kathryn Torok: None declared, Valda Stanevicha: None declared, Jordi Anton Grant/research support from: grants from Pfizer, abbvie, Novartis, Sobi. Gebro, Roche, Novimmune, Sanofi, Lilly, Amgen, Grant/research support from: Pfizer, abbvie, Novartis, Sobi. Gebro, Roche, Novimmune, Sanofi, Lilly, Amgen, Consultant of: Novartis, Sobi, Pfizer, abbvie, Consultant of: Novartis, Sobi, Pfizer, abbvie, Speakers bureau: abbvie, Pfizer, Roche, Novartis, Sobi, Gebro, Speakers bureau: abbvie, Pfizer, Roche, Novartis, Sobi, Gebro, edoardo marrani: None declared, Maria T. Terreri: None declared, Flávio R. Sztajnbok: None declared, Cristina Battagliotti: None declared, Lillemor Berntson Consultant of: paid by Abbvie as a consultant, Speakers bureau: paid by Abbvie for giving speaches about JIA, Despina Eleftheriou: None declared, Gerd Horneff Grant/research support from: AbbVie, Chugai, Merck Sharp & Dohme, Novartis, Pfizer, Roche, Speakers bureau: AbbVie, Bayer, Chugai, Merck Sharp & Dohme, Novartis, Pfizer, Roche, Farzana Nuruzzaman: None declared, Nicola Helmus: None declared

scholarly journals Associations between cardiac and pulmonary involvement in patients with juvenile dermatomyositis—a cross-sectional study

2022 ◽  
Author(s):  
Birgit Nomeland Witczak ◽  
Thomas Schwartz ◽  
Zoltan Barth ◽  
Eli Taraldsrud ◽  
May Brit Lund ◽  
...  
Keyword(s):  
Computed Tomography ◽  
High Resolution ◽  
Cardiac Involvement ◽  
Juvenile Dermatomyositis ◽  
Pulmonary Function Tests ◽  
Pulmonary Involvement ◽  
Left Ventricular ◽  
Organ Involvement ◽  
High Resolution Computed Tomography ◽  
Increased Risk

AbstractThis study aimed at exploring the association between detectable cardiac and pulmonary involvement in long-term juvenile dermatomyositis (JDM) and to assess if patients with cardiac and pulmonary involvement differ with regard to clinical characteristics. 57 JDM patients were examined mean 17.3 (10.5) years after disease onset; this included clinical examination, myositis specific/associated autoantibodies (immunoblot), echocardiography, pulmonary function tests and high-resolution computed tomography. Cardiac involvement was defined as diastolic and/or systolic left ventricular dysfunction and pulmonary involvement as low diffusing capacity for carbon monoxide, low total lung capacity and/or high-resolution computed tomography abnormalities. Patients were stratified into the following four groups: (i) no organ involvement, (ii) pulmonary only, (iii) cardiac only, and (iv) co-existing pulmonary and cardiac involvement. Mean age was 25.7 (12.4) years and 37% were males. One patient had coronary artery disease, seven had a history of pericarditis, seven had hypertension and three had known interstitial lung disease prior to follow-up. There was no association between cardiac (10/57;18%) and pulmonary (41/57;72%) involvement (p = 0.83). After stratifying by organ involvement, 21% of patients had no organ involvement; 61% had pulmonary involvement only; 7% had cardiac involvement only and 11% had co-existing pulmonary or cardiac involvement. Patients with co-existing pulmonary or cardiac involvement had higher disease burden than the remaining patients. Patients with either cardiac or pulmonary involvement only, differed in clinical and autoantibody characteristics. We found no increased risk of developing concomitant cardiac/pulmonary involvement in JDM. Our results shed light upon possible different underlying mechanisms behind pulmonary and cardiac involvement in JDM.

Sex difference in left ventricular global longitudinal strain in patients with systemic sclerosis: association with outcomes

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
T Gegenava ◽  
N Leeuwen ◽  
S Wijngaarden ◽  
J Vries-Bouwstra ◽  
D Cassani ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Longitudinal Strain ◽  
Cardiac Involvement ◽  
Global Longitudinal Strain ◽  
Left Ventricular ◽  
Chi Square ◽  
Female Patients ◽  
All Cause Mortality ◽  
Echocardiographic Parameters ◽  
Males And Females

Abstract Background Cardiac involvement is an important cause of hospitalization and mortality in patients with systemic sclerosis (SSc). Advanced echocardiographic measures such as global longitudinal strain (GLS) have already demonstrated to help identifying cardiac involvement and improve risk-stratification in these patients. However, possible sex differences in echocardiographic parameters including GLS have not been explored so far. Purpose To compare standard and advanced (GLS) echocardiographic parameters between male and female patients with SSc and evaluate their association with cardiovascular outcomes. Methods A total of 408 patients (345 females, 54±14 years old and 63 males 51±13 years old) were included in the study. The study endpoint was all-cause mortality combined with hospitalisations for heart failure, myocardial infarction, coronary interventions, device implantations, arrhythmias, cerebral infarction and peripheral ischemic disease. Results Males and females were comparable in terms of cardiovascular risk-factors and comorbidities but showed differences in terms of disease characteristics: greater modified rodnan skin score and higher creatine phosphokinase was observed in males as compared to females, although high NT-proBNP and deteriorated glomerular filtration rate was more prevalent in females. By standard echocardiography, male SSc patients were characterised by greater left ventricular (LV) volumes, but no difference was observed in LV ejection fraction. By advanced echocardiographic analysis, LV GLS was more preserved in female patients (−21% (IQR: −22% to −20%) as compared to males (−20% (IQR −21% to −19%), p<0.001. After median follow-up of 39 months (IQR: 22–66), the combined endpoint occurred in 84 patients, males were affected significantly more frequently as compared to females (20 (32%) vs. 64 (19%), p=0.017). Kaplan-Meier survival analysis showed that impaired LV GLS (based on median value −20%) was associated with higher cumulative rates of all-cause mortality both in males and females with SSc (females: Chi-Square = 80.307 Log Rank <0.001; males: Chi-Square = 4.493 Log Rank = 0.034) (Fig. 1). In univariate cox regression analyses, LV GLS was also significantly associated with the endpoint both in males and females (in males HR: 1.291, 95% CI: 1.033–1.612, p=0.025, in females HR: 1.386, 95% CI: 1.290–1.491, p<0.001). Conclusions Our study shows that among patients with SSc, LV GLS is more impaired in males as compared to females but in both groups is associated with higher prevalence of death and cardiovascular hospitalization. Funding Acknowledgement Type of funding source: None

P900Electrocardiographic findings in Anderson-Fabry disease versus sarcomeric hypertrophic cardiomyopathy

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
G Vitale ◽  
E Biagini ◽  
M Ziacchi ◽  
F Di Nicola ◽  
M Graziosi ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Cardiac Involvement ◽  
Organ Damage ◽  
Left Ventricular ◽  
St Segment ◽  
Replacement Fibrosis ◽  
Group A ◽  
Group B ◽  
Repolarization Abnormalities ◽  
T Waves

Abstract Background Cardiac involvement is one of the most frequent and disabling organ damage in Anderson-Fabry (AF) disease, causing hypertrophic cardiomyopathy (HCM), conduction disturbances, arrhythmias and coronary disease. Differential diagnosis from sarcomeric HCM is often very challenging, especially for patients with exclusive cardiac involvement. Purpose To gain new insights from standard electrocardiogram (ECG) in AF disease, and identify ECG differences from sarcomeric HCM. Methods Sixty-two consecutive patients (27 males, mean age: 62±16 years) with definite diagnosis of AF disease from 2 Italian centres were evaluated for ECG analysis and divided in 2 groups, according to the presence (Group A, N=39) or the absence (Group B, N=26) of cardiac involvement [hypertrophy detected at echocardiogram or cardiac magnetic resonance (CMR)]. All ECGs were analysed by 2 independent investigators. For Group A, when CMR was performed, a correlation between CMR and ECG was assessed. Patients with cardiac involvement were matched with 78 sarcomeric HCM patients according to sex, age and septal wall thickness on echocardiogram. Results Two AF patients out 39 with cardiac involvement (5%) had normal ECG. Short PR and I degree atrio-ventricular (AV) block were both reported in 6 (15%) cases. Twenty-six (67%) patients showed left ventricular hypertrophy and the majority (85%) had abnormal repolarization. CMR was performed in 22 patients (56%); the 11 (50%) patients with replacement fibrosis had a higher mean Sokolow-Lyon score (4.1±1.8 vs 2.9±1.0 mV; p=0.05), more frequent ST segment depression (82 vs 27%; p=0.03) and negative T waves (91 vs 36%; p=0.027; sensitivity: 90%; specificity: 63%), compared with the 11 without replacement fibrosis. When compared with sarcomeric HCM, AF patients with cardiac involvement had a significantly wider QRS (120±30 vs 100±16 msec; p<0.0001), a higher frequency of right bundle branch block (RBBB) (18 vs 3%; p=0.01), ST segment depression (54 vs 20%; p<0.0001) and negative T waves (72 vs 46%; p=0.01), typically in the inferior leads (44 vs 14%; p<0.0001). No significant differences in terms of pseudo-necrosis and QRS voltages were found. Among Group B AF patients (26, mean age: 36±12 years), 4 had short PR and 5 incomplete RBBB. Four had an abnormal ECG (1 left atrial enlargement, 2 unspecific repolarization abnormalities, 1 Sokolow score of 3.5 mV). Conclusions Standard ECG can detect cardiac involvement in AF disease. A good correlation was reported between repolarization abnormalities and replacement fibrosis on CMR. Wide QRS and RBBB were more frequent among AF patients compared to age-sex-matched sarcomeric HCM ones, probably due to the different aetiology of the diseases (infiltrative disease vs pure hypertrophy).

scholarly journals FRI0237 COMPARISON OF DIFFERENT PULMONARY HYPERTENSION SCREENING ALGORITHMS IN PATIENTS WITH SYSTEMIC SCLEROSIS

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 702.1-703
Author(s):  
M. Erdogan ◽  
B. Kilickiran Avci ◽  
C. Ebren ◽  
Y. Ersoy ◽  
Z. Ongen ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Systemic Sclerosis ◽  
Pulmonary Function Tests ◽  
Left Ventricular ◽  
Systolic Pulmonary Artery Pressure ◽  
Research Support ◽  
Severe Left Ventricular Dysfunction ◽  
Number Of Patients ◽  
Group 3 ◽  
Group 2

Background:Pulmonary hypertension (PH) is an important cause of morbidity and mortality in patients with systemic sclerosis (SSc). Different screening algorithms have been proposed for identifying patients who have a high probability of PH and require right heart catheterization (RHC), which is the gold standard for diagnosing PH.Objectives:To compare the performance of PH screening algorithms in our patients with SSc.Methods:Sixty-nine consecutive pts fulfilling ACR/EULAR 2013 SSc criteria have been screened for PH until now, using the 2015 ESC/ERS, DETECT and ASIG algorithms. Pulmonary function tests (PFT), diffusing capacity of the lung for carbon monoxide (DLCO), trans-thoracic echocardiography, serum NT-proBNP and uric acid assay and high-resolution computed tomography (HRCT) were performed as needed. Patients with known PH, severe interstitial lung disease and severe left ventricular dysfunction (LVD) were not included. RHC was performed in all patients with positive screening according to any one of the screening algorithms. Pts with PH were classified according to the updated PH classification criteria. Sensitivity and specificity of the 3 screening algorithms were evaluated according to the established cut-off value of 25 mmHg for mean systolic pulmonary artery pressure and for the recently proposed cut-off value of 20 mmHg.Results:Among the 69 SSc pts, 27 were excluded due to ILD(n=6), LVD(n=6), already diagnosed PH(n=4) no measurable TRV(n=5), lung cancer (n=2), pulmonary embolism (n=1) and nephrotic syndrome (n=1). Among the remaining 42 patients, 17 required RHC according to at least one of the screening algorithms (Table 1). Number of patients who had suspected pulmonary hypertension and required RHC according to ESC/ERS 2015, DETECT and ASIG were 7 (%17), 13 (%31), and 12 (%29) respectively (Figure 1). Among the 17 pts. who had RHC, PH was present in 3 pts according to the 25-mmHg cut-off (Group 2 in 2, Group 3 in 1) and in 9 pts according to the 20-mmHg cut-off (Group 1 in 5, Group 2 in 3, Group 3 in 1). The sensitivity and specificities were presented in Table 2. ASIG and DETECT had better sensitivity for 25-mmHg cut-off and was better with ASIG for 20 mmHg cut-off. The specificity was better with ESC/ERS for both cut-off values.Conclusion:The ASIG algorithm has a better sensitivity and ESC/ERS algorithm has a better specificity for detecting PH in patients with SSc. A limitation of this study was that RHC was not performed in patients who did not fulfill criteria according to any of the screening algorithms. The sensitivities may be lower than what we propose if there are patients with PH who are asymptomatic and not captured with any of the algorithms.Disclosure of Interests:Mustafa Erdogan: None declared, Burcak Kilickiran Avci: None declared, Cansu Ebren: None declared, Yagmur Ersoy: None declared, Zeki Ongen: None declared, Gul Ongen: None declared, Vedat Hamuryudan Speakers bureau: Pfizer, AbbVie, Amgen, MSD, Novartis, UCB, Gulen Hatemi Grant/research support from: BMS, Celgene Corporation, Silk Road Therapeutics – grant/research support, Consultant of: Bayer, Eli Lilly – consultant, Speakers bureau: AbbVie, Mustafa Nevzat, Novartis, UCB – speaker

scholarly journals Prevalence and factors associated with left ventricular dysfunction in the EULAR Scleroderma Trial and Research group (EUSTAR) database of patients with systemic sclerosis

2009 ◽  
Vol 69 (01) ◽  
pp. 218-221 ◽  
Author(s):  
Y Allanore ◽  
C Meune ◽  
M C Vonk ◽  
P Airo ◽  
E Hachulla ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Calcium Channel ◽  
Multiple Regression ◽  
Calcium Channel Blockers ◽  
Left Ventricular ◽  
Second Phase ◽  
Channel Blockers ◽  
Muscle Involvement ◽  
Factors Associated ◽  
Lv Dysfunction

Objectives:To measure the prevalence of, and factors associated with, left ventricular (LV) dysfunction in systemic sclerosis (SSc).Methods:The EUSTAR database was first searched. A case-control study of a patient subset was then performed to further identify independent factors associated with LV dysfunction by simple and multiple regression.Results:Of 7073 patients, 383 (5.4%) had an LV ejection fraction (EF) of <55%. By multiple regression analysis, age, sex, diffuse cutaneous disease, disease duration, digital ulcerations, renal and muscle involvement, disease activity score, pulmonary fibrosis and pulmonary arterial hypertension were associated with LV dysfunction. In the second phase, 129 patients with SSc with LVEF <55% were compared with 256 patients with SSc with normal LVEF. Male sex (OR 3.48; 95% CI 1.74 to 6.98), age (OR 1.03; 95% CI 1.01 to 1.06), digital ulcerations (OR 1.91; 95% CI 1.05 to 3.50), myositis (OR 2.88; 95% CI 1.15 to 7.19) and use of calcium channel blockers (OR 0.41; 95% CI 0.22 to 0.74) were independent factors associated with LV dysfunction.Conclusion:The prevalence of LV dysfunction in SSc is 5.4%. Age, male gender, digital ulcerations, myositis and lung involvement are independently associated with an increased prevalence of LV dysfunction. Conversely, the use of calcium channel blockers may be protective.

scholarly journals Myocardial native T1 mapping and correlations with clinical and CMR parameters in patients with systemic sclerosis

2021 ◽  
Vol 22 (Supplement_1) ◽  
Author(s):  
A Meloni ◽  
L Gargani ◽  
C Bruni ◽  
C Cavallaro ◽  
M Gobbo ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Myocardial Fibrosis ◽  
Healthy Subjects ◽  
T1 Mapping ◽  
Cardiac Involvement ◽  
Left Ventricular ◽  
Volume Index ◽  
Imaging Protocol ◽  
Native T1 ◽  
Spin Echo Sequence

Abstract Funding Acknowledgements Type of funding sources: None. Background Systemic sclerosis (SSc) is a connective tissue disease characterized by diffuse vascular lesions and fibrosis, also affecting the heart. Cardiovascular magnetic resonance (CMR) can detect replacement myocardial fibrosis by late gadolinium enhancement (LGE) and interstitial myocardial fibrosis/edema by T1 mapping techniques. Purpose To evaluate the prevalence of cardiac involvement by native T1 mapping and its correlation with clinical and CMR parameters in SSc patients. Methods Fifty-one consecutive SSc patients (mean age 51.8 ± 13.7 years, 42 females) and 51 healthy subjects matched for age and sex underwent clinical, bio-humoral assessment, and CMR at 1.5T (Signa Artist, GE Healthcare ). The imaging protocol included: cine, T1 mapping by MOLLI, T2 mapping by multi-echo fast-spin-echo sequence, LGE, and STIR T2-weighted sequences. Native T1 and T2 values were assessed in all 16 myocardial segments and the global value was the mean. Results. Global native T1 values were significantly higher in SSc patients than in healthy subjects (1076.4 ± 50.7 vs 1033.3 ± 31.9 ms; P &lt; 0.0001). As in healthy subjects, in patients native T1 values were significantly lower in males than in females (1033.4 ± 38.3 vs 1085.6 ± 48.6 ms; P = 0.004) and inversely correlated with age (R=-0415; P = 0.002). Twenty-three (45.1%) patients had an increased global heart T1 value (&gt;1060 ms in males and &gt;1085 ms in females). Of them, 14 patients (60.9 %) showed positive LGE. Frequency of cardiovascular risk factors, indices of disease activity and chronicity, biochemical parameters, and cardio-active therapy were comparable between patients with normal and elevated T1. Compared to patients with normal T1 value, patients with elevated T1 had significantly higher left ventricular (LV) end-diastolic volume index (76.8 ± 13.3 vs 69.2 ± 11.8, P = 0.050), LV stroke volume index (49.7 ± 6.4 vs 44.4 ± 6.9 ml/m2; P = 0.010), LV cardiac output (3.6 ± 0.5  vs 3.0 ± 0.6 l/min /m2; P &lt; 0.0001), and global heart T2 values (60.1 ± 3.6 vs 55.7 ± 3.1 ms; P &lt; 0.0001). Replacement myocardial fibrosis was detected in 24 (47.1%) patients and they showed significantly higher global heart native T1 values (Figure 1A). Positive T2-weighted images for myocardial oedema were found in 5 (9.8%) patients, all with increased global heart native T1 value. Patients with oedema had significantly higher native global heart T1 values (Figure 1B). Conclusion Elevated native T1 values measured by CMR are frequent in SSc patients and they are associated with inflammation, replacement fibrosis, and increased LV dimension. CMR T1 mapping seems to be a sensitive parameter to include in the routine clinical assessment of SSc patients for detecting earlier pejorative cardiac involvement, although prospective data are recommended. Abstract Figure.

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