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Heart involvement in systemic sclerosis: a combined echocardiographic and scintigraphic study

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Abstract

The aim of this study is to investigate systemic sclerosis (SSc) patients without clinically evident heart disease for cardiac abnormalities. SSc patients and age- and sex-matched healthy controls from the hospital staff underwent transthoracic echocardiography for the assessment of the left ventricle (LV) morphology and function and estimation of the pulmonary artery systolic pressure (PASP). Patients further underwent stress-rest myocardial perfusion imaging (MPI) scintigraphy by single-photon emission computed tomography (SPECT). Thirty-seven patients were included (33 women, 19 with diffuse, and 18 with limited SSc). LV hypertrophy was more common in SSc patients than controls (24.3 vs 0 %, p = 0.001). Impaired LV relaxation was found in 45.9 % of patients and 40.5 % controls (p = 0.639). Excluding patients with arterial hypertension, LV hypertrophy was still found in 23.1 % and LV relaxation impairment in 38.5 %. PASP over 30 mmHg was found in 13 patients (35.1 %), 11 of whom had no history of pulmonary arterial hypertension (PAH). Of 35 patients who underwent SPECT, 21 patients (60 %) exhibited reversible LV perfusion defects. Their mean age was 51.8 years; four patients were younger than 40 years old and eight patients younger than 50 years. In all cases, ischemia was graded as mild or moderate and in a single case, graded as significant. Subclinical heart involvement is common in SSc patients even in the younger age groups. LV hypertrophy and impaired relaxation, raised PASP, and ischemia on MPI with SPECT are found in a significant proportion of SSc patients. Careful screening of SSc patients for potential heart involvement and consultation by a cardiologist may be of value.

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References

  1. Geyer M, Müller-Ladner U (2011) The pathogenesis of systemic sclerosis revisited. Clin Rev Allergy Immunol 40:92–103

    Article  PubMed  Google Scholar 

  2. Liakouli V, Cipriani P, Marrelli A, Alvaro S, Ruscitti P, Giacomelli R (2011) Angiogenic cytokines and growth factors in systemic sclerosis. Autoimmun Rev 10:590–594

    Article  CAS  PubMed  Google Scholar 

  3. Baraut J, Michel L, Verrecchia F, Farge D (2010) Relationship between cytokine profiles and clinical outcomes in patients with systemic sclerosis. Autoimmun Rev 10:65–73

    Article  CAS  PubMed  Google Scholar 

  4. Ioannidis JP, Vlachoyiannopoulos PG, Haidich AB, Medsger TA Jr, Lucas M, Michet CJ, Kuwana M, Yasuoka H, van den Hoogen F, Te Boome L, van Laar JM, Verbeet NL, Matucci-Cerinic M, Georgountzos A, Moutsopoulos HM (2005) Mortality in systemic sclerosis: an international meta-analysis of individual patient data. Am J Med 118:2–10

    Article  PubMed  Google Scholar 

  5. Follansbee WP, Miller TR, Curtiss EI, Orie JE, Bernstein RL, Kiernan JM, Medsger TA Jr (1990) A controlled clinicopathologic study of myocardial fibrosis in systemic sclerosis (scleroderma). J Rheumatol 17:656–662

    CAS  PubMed  Google Scholar 

  6. Khurma V, Meyer C, Park GS, McMahon M, Lin J, Singh RR, Khanna D (2008) A pilot study of subclinical coronary atherosclerosis in systemic sclerosis: coronary artery calcification in cases and controls. Arthritis Rheum 59:591–597

    Article  CAS  PubMed  Google Scholar 

  7. Montisci R, Vacca A, Garau P, Colonna P, Ruscazio M, Passiu G, Iliceto S, Mathieu A (2003) Detection of early impairment of coronary flow reserve in patients with systemic sclerosis. Ann Rheum Dis 62:890–893

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  8. Sulli A, Ghio M, Bezante GP, Deferrari L, Craviotto C, Sebastiani V, Setti M, Barsotti A, Cutolo M, Indiveri F (2004) Blunted coronary flow reserve in systemic sclerosis. Rheumatology (Oxford) 43:505–509

    Article  CAS  Google Scholar 

  9. Akram MR, Handler CE, Williams M, Carulli MT, Andron M, Black CM, Denton CP, Coghlan JG (2006) Angiographically proven coronary artery disease in scleroderma. Rheumatology (Oxford) 45:1395–1398

    Article  CAS  Google Scholar 

  10. Tsifetaki N, Georgiadis AN, Alamanos Y, Fanis S, Argyropoulou MI, Drosos AA (2010) Subclinical atherosclerosis in scleroderma patients. Scand J Rheumatol 39:326–329

    Article  CAS  PubMed  Google Scholar 

  11. Kuznetsova T, Herbots L, López B, Jin Y, Richart T, Thijs L, González A, Herregods MC, Fagard RH, Díez J, Staessen JA (2009) Prevalence of left ventricular diastolic dysfunction in a general population. Circ Heart Fail 2:105–112

    Article  PubMed  Google Scholar 

  12. Allanore Y, Meune C, Vonk MC, Airo P, Hachulla E, Caramaschi P, Riemekasten G, Cozzi F, Beretta L, Derk CT, Komócsi A, Farge D, Balbir A, Riccieri V, Distler O, Chialà A, Papa ND, Simic KP, Ghio M, Stamenkovic B, Rednic S, Host N, Pellerito R, Zegers E, Kahan A, Walker UA, Matucci-Cerinic M; EUSTAR co-authors (2010) Prevalence and factors associated with left ventricular dysfunction in the EULAR Scleroderma Trial and Research group (EUSTAR) database of patients with systemic sclerosis. Ann Rheum Dis 69:218-21

  13. Meune C, Avouac J, Wahbi K, Cabanes L, Wipff J, Mouthon L, Guillevin L, Kahan A, Allanore Y (2008) Cardiac involvement in systemic sclerosis assessed by tissue-Doppler echocardiography during routine care: a controlled study of 100 consecutive patients. Arthritis Rheum 58:1803–1809

    Article  PubMed  Google Scholar 

  14. Dimitroulas T, Giannakoulas G, Papadopoulou K, Karvounis H, Dimitroula H, Koliakos G, Karamitsos T, Parcharidou D, Settas L (2010) Early detection of cardiac involvement in systemic sclerosis assessed by tissue-Doppler echocardiography: relationship with neurohormonal activation and endothelial dysfunction. J Rheumatol 37:993–999

    Article  PubMed  Google Scholar 

  15. Walker UA, Tyndall A, Czirják L, Denton C, Farge-Bancel D, Kowal-Bielecka O, Müller-Ladner U, Bocelli-Tyndall C, Matucci-Cerinic M (2007) Clinical risk assessment of organ manifestations in systemic sclerosis: a report from the EULAR Scleroderma Trials And Research group database. Ann Rheum Dis 66:754–763

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  16. de Groote P, Gressin V, Hachulla E, Carpentier P, Guillevin L, Kahan A, Cabane J, Francès C, Lamblin N, Diot E, Patat F, Sibilia J, Petit H, Cracowski JL, Clerson P, Humbert M, ItinerAIR-Scleroderma Investigators (2008) Evaluation of cardiac abnormalities by Doppler echocardiography in a large nationwide multicentric cohort of patients with systemic sclerosis. Ann Rheum Dis 67:31–36

    Article  PubMed  Google Scholar 

  17. Fernandes F, Ramires FJ, Arteaga E, Ianni BM, Bonfá ES, Mady C (2003) Cardiac remodeling in patients with systemic sclerosis with no signs or symptoms of heart failure: an endomyocardial biopsy study. J Card Fail 9:311–317

    Article  PubMed  Google Scholar 

  18. Hachulla AL, Launay D, Gaxotte V, de Groote P, Lamblin N, Devos P, Hatron PY, Beregi JP, Hachulla E (2009) Cardiac magnetic resonance imaging in systemic sclerosis: a cross-sectional observational study of 52 patients. Ann Rheum Dis 68:1878–1884

    Article  PubMed Central  PubMed  Google Scholar 

  19. Tzelepis GE, Kelekis NL, Plastiras SC, Mitseas P, Economopoulos N, Kampolis C, Gialafos EJ, Moyssakis I, Moutsopoulos HM (2007) Pattern and distribution of myocardial fibrosis in systemic sclerosis: a delayed enhanced magnetic resonance imaging study. Arthritis Rheum 56:3827–3836

    Article  PubMed  Google Scholar 

  20. Follansbee WP, Curtiss EI, Medsger TA Jr, Steen VD, Uretsky BF, Owens GR, Rodnan GP (1984) Physiologic abnormalities of cardiac function in progressive systemic sclerosis with diffuse scleroderma. N Engl J Med 310:142–148

    Article  CAS  PubMed  Google Scholar 

  21. Follansbee WP, Curtiss EI, Medsger TA Jr, Owens GR, Steen VD, Rodnan GP (1984) Myocardial function and perfusion in the CREST syndrome variant of progressive systemic sclerosis. Exercise radionuclide evaluation and comparison with diffuse scleroderma. Am J Med 77:489–496

    Article  CAS  PubMed  Google Scholar 

  22. Gustafsson R, Mannting F, Kazzam E, Waldenström A, Hällgren R (1989) Cold-induced reversible myocardial ischaemia in systemic sclerosis. Lancet 2:475–479

    Article  CAS  PubMed  Google Scholar 

  23. Alexander EL, Firestein GS, Weiss JL, Heuser RR, Leitl G, Wagner HN Jr, Brinker JA, Ciuffo AA, Becker LC (1986) Reversible cold-induced abnormalities in myocardial perfusion and function in systemic sclerosis. Ann Intern Med 105:661–668

    Article  CAS  PubMed  Google Scholar 

  24. Bulkley BH, Ridolfi RL, Salyer WR (1976) Hutchins GM (2007) Myocardial lesions of progressive systemic sclerosis. A cause of cardiac dysfunction. Circulation 53:483–490

    Article  CAS  PubMed  Google Scholar 

  25. McMahon M, Hahn BH. Atherosclerosis and systemic lupus erythematosus: mechanistic basis of the association. Curr Opin Immunol 19:633-9

  26. Kitas GD, Gabriel SE (2011) Cardiovascular disease in rheumatoid arthritis: state of the art and future perspectives. Ann Rheum Dis 70:8–14

    Article  PubMed  Google Scholar 

  27. Steen VD, Follansbee WP, Conte CG, Medsger TA Jr (1996) Thallium perfusion defects predict subsequent cardiac dysfunction in patients with systemic sclerosis. Arthritis Rheum 39:677–681

    Article  CAS  PubMed  Google Scholar 

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Correspondence to Alexandros A. Drosos.

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Papagoras, C., Achenbach, K., Tsifetaki, N. et al. Heart involvement in systemic sclerosis: a combined echocardiographic and scintigraphic study. Clin Rheumatol 33, 1105–1111 (2014). https://doi.org/10.1007/s10067-014-2666-3

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  • DOI: https://doi.org/10.1007/s10067-014-2666-3

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