Incidence and Predictors of Severe Heart Disease in Systemic Sclerosis

Serena Vettori¹, Yannick Allanore², Laszlo Czirjak³, Francesco Del Galdo⁴, Christopher P. Denton⁵, Oliver Distler⁶, Ivan Foeldvari⁷, Marc Frerix⁸, Veronika K. Jaeger⁹, Brigitte Krummel-Lorenz¹⁰, Marco Matucci Cerinic¹¹, Carina Mihai¹², Ulf Müller-Ladner¹³, Gabriela Riemekasten¹⁴, Tim Schmeiser¹⁵, Ingo H. Tarner⁸, Ulrich A. Walker⁹, Gabriele Valentini¹⁶ and EUSTAR Group and DeSScipher Consortium, ¹Department of Internal and Experimental Medicine, Rheumatology Unit, Second University of Naples, Naples, Italy, ²Department of Rheumatology, University Paris Descartes and Cochin Hospital, Paris, France, ³Rheumatology and Immunology Medical School, University of Pécs, Pécs, Hungary, ⁴Division of Musculoskeletal Diseases, University of Leeds, Leeds, United Kingdom, ⁵Department of Rheumatology, Royal Free and University College Medical School, London, United Kingdom, ⁶Department of Rheumatology and Center of Experimental Rheumatology, University Hospital Zurich, Zurich, Switzerland, ⁷Hamburg Center for Pediatric and Adolescent Rheumatology, Hamburg, Germany, ⁸Justus-Liebig-University of Giessen, Kerckhoff-Klinik, Bad Nauheim, Germany, ⁹Department of Rheumatology, University Hospital Basel, Basel, Switzerland, ¹⁰Rheumatologist, Frankfurt, Germany, ¹¹Department of Internal Medicine, Division of Rheumatology, University of Florence, Florence, Italy, ¹²Internal Medicine and Rheumatology Departement, Cantacuzino Hospital, Carol Davila University of Medicine and Pharmacy, Bucharest, Romania, Bucharest, Romania, ¹³Internal Medicine and Rheumatology, Justus-Liebig-University of Giessen, Kerckhoff-Klinik, Bad Nauheim, Germany, ¹⁴Rheumatology and Clinical Immunology, Charité – University Hospital, Berlin, Berlin, Germany, ¹⁵Internistic Rheumatology, Krankenhaus St. Josef, Wuppertal, Germany, ¹⁶Internal and Experimental Medicine, Rheumatology Unit, Second University of Naples, Naples, Italy

Meeting: 2015 ACR/ARHP Annual Meeting

Date of first publication: September 29, 2015

Keywords: heart disease and systemic sclerosis

Session Information

Date: Sunday, November 8, 2015

Title: Systemic Sclerosis, Fibrosing Syndromes and Raynaud's - Clinical Aspects and Therapeutics Poster I

Session Type: ACR Poster Session A

Session Time: 9:00AM-11:00AM

Background/Purpose: Heart disease occurs in most patients with systemic sclerosis (SSc), as emerged by autopsy and imaging studies. It can cause cardiac blocks (CBs), ventricular arrhythmias (VA), Q waves, and congestive heart failure (CHF), referred to as “severe heart disease” (SHD), as they may require a pacemaker/defibrillator implant (PM/DF) or ensue in death/sudden death (SD) in about 20% of cases. In a retrospective study, SHD has been recently found in 211/1119 SSc patients from the European Scleroderma Trial and Research (EUSTAR) database at first visits. We aimed to look for SHD incidence in a large SSc cohort and to identify SHD-related predictors.

Methods: SSc patients prospectively enrolled by 14 centers into the EUSTAR database from April 16 2013 to April 30 2015 with at least 1 follow-up (FU) visit were analyzed. At baseline, the prevalence of SHD manifestations was calculated. Incidence rates of SHD manifestations and their associations with demographics, disease features, and vasodilatory treatment were also calculated (study protocol at www.clinicaltrials.gov; NCT01836263).

Results:

Out of 449 patients meeting the new ACR/EULAR criteria for SSc with complete data on SHD, 284 (63%) had at least 1 FU visit (median FU 1.01 years, range 0.18-1.99) and were analyzed. Eighty-seven % were females; median age was 57 years (range 13-86); median disease duration 8 (range 0-61); 26% had diffuse cutaneous SSc; 74% limited cutaneous SSc. ANA were positive in 98% of patients (42% ACA; 34% anti-Scl-70; 5% anti-RNA polymeraseIII; 3% anti-PmScl; 3% anti-U1RNP). At baseline, 65/284 (23%) patients had at least 1 SHD manifestation: 52/280 (19%) had CBs, 2/267(1%) VA, 9/269 (3%) CHF, 6/262 (2%) Q waves, and PM/DF had been applied in 3/269 (1%). There was no statistical difference in any demographic and disease feature nor in the prevalence of SHD manifestations between the whole cohort and the patients investigated. During FU, 32 patients had a new SHD manifestation (20 CBs, 7 VA, 4 CHF, 2 Q waves, 1 PM/DF), with incidence rates of 11/100 patient*year for any SHD, 7/100 patient*year for CBs, 2.5/100 patient*year for VA, 1.43/100 patient*year for CHF, 0.7/100 patient*year for Q waves, and 0.4/100 patient*year for PM/DF. In univariate analysis, newly developed SHD manifestations were associated with baseline SHD, dyspnea, chest X-ray fibrosis, FVC and DLCO<80% of predicted, echo-assessed pulmonary hypertension (PH), modified Rodnan Skin Score (mRSS) and active disease (European Scleroderma Study Group Activity Index). At multivariate Cox-regression analysis, mRSS (HR 1.1, 95%CI 1.03-1.21) and echo-assessed PH (HR 17, 95%CI 4.4-66) were independent predictors of new SHD manifestations. New cases of CHF were only observed in patients with preexisting SHD.

Conclusion: To our knowledge, this is the first study investigating the incidence of all SHD manifestations in SSc. Our results suggest that mRss and echocardiographic PH mark an increased risk of new SHD manifestations over time and that CHF only develops in patients with preexisting SHD.

Aknowledgements. Funded by the European Community’s Framework Programme 7 (FP7-HEALTH-2012.2.4.4-2 – Observational trials in rare diseases); grant n. 305495.

Disclosure: S. Vettori, None; Y. Allanore, Actelion, Bayer, Biogen, Bristol-Meyers Squibb, Inventiva, Medac, Pfizer, Roche/Genentech, Sanofi-Aventis, Servier, 2,Actelion, Bayer, Biogen, Bristol-Meyers Squibb, Inventiva, Medac, Pfizer, Roche/Genentech, Sanofi-Aventis, Servier, 5; L. Czirjak, None; F. Del Galdo, None; C. P. Denton, GlaxoSmithKline, 2,Actelion Pharmaceuticals US, 5,GlaxoSmithKline, 5,Serono, 5,Inventiva, 5,CSL Behring, 2,Bayer, 5; O. Distler, Consultancy relationships and/or has received research funding from Actelion, Pfizer, Ergonex, BMS, Sanofi-Aventis, United BioSource Corporation, Medac, Biovitrium, Boehringer Ingelheim, Bayer Pharma AG, Novartis, 4D Science and Active Biotech in the area, 2; I. Foeldvari, None; M. Frerix, None; V. K. Jaeger, None; B. Krummel-Lorenz, None; M. Matucci Cerinic, None; C. Mihai, None; U. Müller-Ladner, None; G. Riemekasten, None; T. Schmeiser, None; I. H. Tarner, None; U. A. Walker, None; G. Valentini, None.

To cite this abstract in AMA style:

Vettori S, Allanore Y, Czirjak L, Del Galdo F, Denton CP, Distler O, Foeldvari I, Frerix M, Jaeger VK, Krummel-Lorenz B, Matucci Cerinic M, Mihai C, Müller-Ladner U, Riemekasten G, Schmeiser T, Tarner IH, Walker UA, Valentini G. Incidence and Predictors of Severe Heart Disease in Systemic Sclerosis [abstract]. Arthritis Rheumatol. 2015; 67 (suppl 10). https://acrabstracts.org/abstract/incidence-and-predictors-of-severe-heart-disease-in-systemic-sclerosis/. Accessed .

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