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Cardiac involvement in systemic sclerosis: differences between clinical subsets and influence on survival

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An Erratum to this article was published on 04 April 2016

Abstract

Cardiac involvement (CI) is a known complication of SSc associated with increased mortality. Our objective was to describe a cohort of patients with SSc and CI and to assess the differences between cutaneous subsets regarding their presentation and survival. Three hundred and ninety-three Spanish patients from a single center, diagnosed with SSc, were retrospectively studied for evidence of CI using noninvasive and invasive tests from 1976 to 2011. Clinical, epidemiological, immunological and therapeutic features of patients with CI were compared to those without it and within the different cutaneous subsets of SSc. CI was present in 173 (44 %) patients. Mitral regurgitation (67 %), conduction alterations (45 %) and left ventricle diastolic dysfunction (40 %) were the most common findings. Pericardial involvement and heart failure were more frequent in diffuse SSc (dcSSc) than in limited or sine scleroderma SSc. CI accounted for 20 % of deaths, and it was an independent mortality risk factor (HR 2.1, P = 0.02), but once CI was established, classical dcSSc mortality risk factors determined mortality. Patients with dcSSc developed CI faster than limited (HR 1.9, P = 0.003) or sine SSc patients (HR 2.5, P = 0.002), specially during the first year after SSc onset. We found statistically significant differences between the 3 SSc subsets in the presentation of pericardial involvement and heart failure. CI increased the mortality and appeared at a higher rate, especially during the first year after SSc onset. Screening for heart involvement should be performed at diagnosis and during follow-up.

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Acknowledgments

We would like to thank Dr. Jaume Candell-Riera (Cardiology Department, Vall d’Hebron University Hospital, Universitat Autònoma de Barcelona, Barcelona, Spain) for his substantial contributions to this work.

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Correspondence to Andreu Fernández-Codina.

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An erratum to this article is available at http://dx.doi.org/10.1007/s00296-016-3470-y.

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Figure 4

Kaplan Meier curves and hazard ratios for survival depending on cardiac involvement in patients diagnosed with SSc before 1990. Survival probability and the number of participants who were at risk at the beginning of each interval are shown below the graph. (TIFF 6172 kb)

Figure 5

Kaplan Meier curves and hazard ratios for survival depending on cardiac involvement in patients diagnosed with SSc after 1990. Survival probability and the number of participants who were at risk at the beginning of each interval are shown below the graph (TIFF 6160 kb)

Figure 6

Kaplan Meier curves and hazard ratios for survival in patients with SSc diagnosed before 1990 and cardiac involvement divided by cutaneous subsets. Survival probability and the number of participants who were at risk at the beginning of each interval are shown below the graph. (TIFF 6162 kb)

Figure 7

Kaplan Meier curves and hazard ratios for survival in patients with SSc diagnosed after 1990 and cardiac involvement divided by cutaneous subsets. Survival probability and the number of participants who were at risk at the beginning of each interval are shown below the graph. (TIFF 6151 kb)

Figure 8

Kaplan Meier curves for the rate of developing cardiac involvement since the onset of SSc divided by cutaneous subsets in patients diagnosed with SSc before 1990. Survival probability and the number of participants who were at risk at the beginning of each interval are shown below the graph. (TIFF 6197 kb)

Figure 9

Kaplan Meier curves for the rate of developing cardiac involvement since the onset of SSc divided by cutaneous subsets in patients diagnosed with SSc after 1990. Survival probability and the number of participants who were at risk at the beginning of each interval are shown below the graph. (TIFF 6194 kb)

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Fernández-Codina, A., Simeón-Aznar, C.P., Pinal-Fernandez, I. et al. Cardiac involvement in systemic sclerosis: differences between clinical subsets and influence on survival. Rheumatol Int 37, 75–84 (2017). https://doi.org/10.1007/s00296-015-3382-2

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