open access

Vol 5, No 4 (2019)
Prace poglądowe
Published online: 2020-02-05
Get Citation

Heart in systemic sclerosis — pathogenesis and diagnosis based on new imaging methods

Żaneta Smoleńska, Ewa Puzio, Karolina Dorniak, Marta Grono-Burakowska, Zbigniew Zdrojewski
DOI: 10.5603/FR.2019.0020
·
Forum Reumatologiczne 2019;5(4):181-189.

open access

Vol 5, No 4 (2019)
Prace poglądowe
Published online: 2020-02-05

Abstract

The heart is one of the most frequently involved organs in systemic sclerosis. Myocardial fibrosis is the most common pathological change; it is caused by changes in microcirculation, inflammation and repair processes. In most systemic sclerosis patients, the course of cardiac involvement is subclinical, especially in the early stages of the disease. This article discusses the mechanisms which lead to cardiac injury, its various clinical forms as well as procedures with respect to diagnosis, with a particular emphasis on new tools, such as echocardiography and cardiac magnetic resonance.

Forum Reumatol. 2019, tom 5, nr 4: 181–189

Abstract

The heart is one of the most frequently involved organs in systemic sclerosis. Myocardial fibrosis is the most common pathological change; it is caused by changes in microcirculation, inflammation and repair processes. In most systemic sclerosis patients, the course of cardiac involvement is subclinical, especially in the early stages of the disease. This article discusses the mechanisms which lead to cardiac injury, its various clinical forms as well as procedures with respect to diagnosis, with a particular emphasis on new tools, such as echocardiography and cardiac magnetic resonance.

Forum Reumatol. 2019, tom 5, nr 4: 181–189

Get Citation

Keywords

systemic sclerosis; cardiac involvement; diagnostic methods

About this article
Title

Heart in systemic sclerosis — pathogenesis and diagnosis based on new imaging methods

Journal

Forum Reumatologiczne

Issue

Vol 5, No 4 (2019)

Pages

181-189

Published online

2020-02-05

DOI

10.5603/FR.2019.0020

Bibliographic record

Forum Reumatologiczne 2019;5(4):181-189.

Keywords

systemic sclerosis
cardiac involvement
diagnostic methods

Authors

Żaneta Smoleńska
Ewa Puzio
Karolina Dorniak
Marta Grono-Burakowska
Zbigniew Zdrojewski

References (50)
  1. Desbois AC, Cacoub P. Systemic sclerosis: An update in 2016. Autoimmun Rev. 2016; 15(5): 417–426.
  2. Kruszec A, Kotyla P. [Heart involvement in systemic sclerosis]. Pol Merkur Lekarski. 2016; 41(243): 156–159.
  3. Meune C, Vignaux O, Kahan A, et al. Heart involvement in systemic sclerosis: evolving concept and diagnostic methodologies. Arch Cardiovasc Dis. 2010; 103(1): 46–52.
  4. D'Angelo WA, Fries JF, Masi AT, et al. Pathologic observations in systemic sclerosis (scleroderma). A study of fifty-eight autopsy cases and fifty-eight matched controls. Am J Med. 1969; 46(3): 428–440.
  5. Steen V, Medsger T. Severe organ involvement in systemic sclerosis with diffuse scleroderma. Arthritis & Rheumatism. 2001; 43(11): 2437–2444, doi: 10.1002/1529-0131(200011)43:11<2437::aid-anr10>3.0.co;2-u.
  6. Frech TM, Revelo MP, Ryan JJ, et al. Cardiac metabolomics and autopsy in a patient with early diffuse systemic sclerosis presenting with dyspnea: a case report. J Med Case Rep. 2015; 9: 136.
  7. Bose N, Chiesa-Vottero A, Chatterjee S. Scleroderma renal crisis. Semin Arthritis Rheum. 2015; 44(6): 687–694.
  8. Saunders WB, Follansbee WP, Marroquin OC. Cardiac involvement in systemic sclerosis. In: Clemens PJ, Furst DE. ed. Systemic Sclerosis. Lippincott Williams & Wilkins, Philadelphia, PA, USA 2004: 195–220.
  9. Mavrogeni SI, Bratis K, Karabela G, et al. Cardiovascular Magnetic Resonance Imaging clarifies cardiac pathophysiology in early, asymptomatic diffuse systemic sclerosis. Inflamm Allergy Drug Targets. 2015; 14(1): 29–36.
  10. Mavrogeni S, Sfikakis PP, Karabela G, et al. Cardiovascular magnetic resonance imaging in asymptomatic patients with connective tissue disease and recent onset left bundle branch block. Int J Cardiol. 2014; 171(1): 82–87.
  11. Allanore Y, Meune C. Primary myocardial involvement in systemic sclerosis: evidence for a microvascular origin. Clin Exp Rheumatol. 2010; 28(5 Suppl 62): S48–S53.
  12. Kahan A, Allanore Y. Primary myocardial involvement in systemic sclerosis. . Rheumatology. 2006; 45(1): 14–17.
  13. Fernández-Codina A, Simeón-Aznar CP, Pinal-Fernandez I, et al. Cardiac involvement in systemic sclerosis: differences between clinical subsets and influence on survival. Rheumatol Int. 2017; 37(1): 75–84.
  14. Meune C, Allanore Y, Devaux JY, et al. High prevalence of right ventricular systolic dysfunction in early systemic sclerosis. J Rheumatol. 2004; 31(10): 1941–1945.
  15. Medsger TA. Jr, Silman AJ, Steen VD, Black CM, Akesson A, Bacon PA, Harris CA, Jablonska S, Jayson MI, Jimenez SA, Krieg T, Leroy EC, Maddison PJ, Russell ML, Schachter RK, Wollheim FA, Zacharaie H. A disease severity scale for systemic sclerosis: development and testing. J Rheumatol. 1999; 26: 2159–2167.
  16. Psarras A, Soulaidopoulos S, Garyfallos A, et al. A critical view on cardiovascular risk in systemic sclerosis. Rheumatol Int. 2017; 37(1): 85–95.
  17. Ferri C, Giuggioli D, Sebastiani M, et al. Heart involvement and systemic sclerosis. Lupus. 2005; 14(9): 702–707.
  18. Boueiz A, Mathai SC, Hummers LK, et al. Cardiac complications of systemic sclerosis: recent progress in diagnosis. Curr Opin Rheumatol. 2010; 22(6): 696–703.
  19. Bournia VK, Tountas C, Protogerou A, et al. Update on assessment and management of primary cardiac involvement in systemic sclerosis. Journal of Scleroderma and Related Disorders. 2018; 3(1): 53–65.
  20. Bissell LA, Md Yusof MdY, Buch MH. Primary myocardial disease in scleroderma-a comprehensive review of the literature to inform the UK Systemic Sclerosis Study Group cardiac working group. Rheumatology (Oxford). 2017; 56(6): 882–895.
  21. Gustafsson R, Mannting F, Kazzam E, et al. Cold-induced reversible myocardial ischaemia in systemic sclerosis. Lancet. 1989; 2(8661): 475–479.
  22. Vacca A, Montisci R, Garau P, et al. Prognostic impact of coronary microcirculation abnormalities in systemic sclerosis: a prospective study to evaluate the role of non-invasive tests. Arthritis Res Ther. 2013; 15(1): R8.
  23. Ntusi NAB, Piechnik SK, Francis JM, et al. Subclinical myocardial inflammation and diffuse fibrosis are common in systemic sclerosis--a clinical study using myocardial T1-mapping and extracellular volume quantification. J Cardiovasc Magn Reson. 2014; 16: 21.
  24. Mavrogeni S, Markousis-Mavrogenis G, Kolovou G. The Sphinx's riddle: cardiovascular involvement in autoimmune rheumatic disease. BMC Cardiovasc Disord. 2016; 16(1): 204.
  25. Cannarile F, Valentini V, Mirabelli G, et al. Cardiovascular disease in systemic sclerosis. Ann Transl Med. 2015; 3(1): 8.
  26. Kerr JR. Pathogenesis of human parvovirus B19 in rheumatic disease. Ann Rheum Dis. 2000; 59(9): 672–683.
  27. Pieroni M, De Santis M, Zizzo G, et al. Recognizing and treating myocarditis in recent-onset systemic sclerosis heart disease: potential utility of immunosuppressive therapy in cardiac damage progression. Semin Arthritis Rheum. 2014; 43(4): 526–535.
  28. Tyndall AJ, Bannert B, Vonk M, et al. Causes and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database. Ann Rheum Dis. 2010; 69(10): 1809–1815.
  29. Draeger HT, Assassi S, Sharif R, et al. Right bundle branch block: a predictor of mortality in early systemic sclerosis. PLoS One. 2013; 8(10): e78808.
  30. Kahan A, Coghlan G, McLaughlin V. Cardiac complications of systemic sclerosis. Rheumatology (Oxford). 2009; 48 Suppl 3: iii45–iii48.
  31. Allanore Y, Meune C, Vonk MC, et al. EUSTAR co-authors. 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. 2010; 69(1): 218–221.
  32. Mavrogeni S, Markousis-Mavrogenis G, Koutsogeorgopoulou L, et al. Cardiovascular magnetic resonance imaging: clinical implications in the evaluation of connective tissue diseases. J Inflamm Res. 2017; 10: 55–61.
  33. Varga-Szemes A, Meinel FG, De Cecco CN, et al. CT myocardial perfusion imaging. AJR Am J Roentgenol. 2015; 204(3): 487–497.
  34. Meune C, Avouac J, Wahbi K, et al. Cardiac involvement in systemic sclerosis assessed by tissue-doppler echocardiography during routine care: A controlled study of 100 consecutive patients. Arthritis Rheum. 2008; 58(6): 1803–1809.
  35. Pellerin D, Sharma R, Elliott P, et al. Tissue Doppler, strain, and strain rate echocardiography for the assessment of left and right systolic ventricular function. Heart. 2003; 89 Suppl 3: iii9–ii17.
  36. Wang M, Yip GWK, Wang AYM, et al. Peak early diastolic mitral annulus velocity by tissue Doppler imaging adds independent and incremental prognostic value. J Am Coll Cardiol. 2003; 41(5): 820–826.
  37. Saeed M, Van TA, Krug R, et al. Cardiac MR imaging: current status and future direction. Cardiovasc Diagn Ther. 2015; 5: 290–310.
  38. Saeed M, Liu H, Liang CH, et al. Magnetic resonance imaging for characterizing myocardial diseases. Int J Cardiovasc Imaging. 2017; 33(9): 1395–1414.
  39. Oda S, Utsunomiya D, Nakaura T, et al. Identification and Assessment of Cardiac Amyloidosis by Myocardial Strain Analysis of Cardiac Magnetic Resonance Imaging. Circ J. 2017; 81(7): 1014–1021.
  40. Stanton KM, Ganigara M, Corte P, et al. The Utility of Cardiac Magnetic Resonance Imaging in the Diagnosis of Cardiac Sarcoidosis. Heart Lung Circ. 2017; 26(11): 1191–1199.
  41. Daniłowicz-Szymanowicz L, Dorniak K, Szurowska E, et al. Re: Assessment of coronary microvascular dysfunction in hypertrophic cardiomyopathy: first-pass myocardial perfusion cardiovascular magnetic resonance imaging at 1.5 T. Clin Radiol. 2014; 69(3): e163–e164.
  42. Tzelepis GE, Kelekis NL, Plastiras SC, et al. Pattern and distribution of myocardial fibrosis in systemic sclerosis: a delayed enhanced magnetic resonance imaging study. Arthritis Rheum. 2007; 56(11): 3827–3836.
  43. Mavrogeni SI, Schwitter J, Gargani L, et al. Cardiovascular magnetic resonance in systemic sclerosis: "Pearls and pitfalls". Semin Arthritis Rheum. 2017; 47(1): 79–85.
  44. Kahan A, Coghlan G, McLaughlin V. Cardiac complications of systemic sclerosis. Rheumatology (Oxford). 2009; 48 Suppl 3: iii45–iii48.
  45. Krumm P, Mueller KAL, Klingel K, et al. Cardiovascular magnetic resonance patterns of biopsy proven cardiac involvement in systemic sclerosis. J Cardiovasc Magn Reson. 2016; 18(1): 70.
  46. Mavrogeni S, Apostolou D, Argyriou P, et al. T1 and T2 Mapping in Cardiology: "Mapping the Obscure Object of Desire". Cardiology. 2017; 138(4): 207–217.
  47. Kobayashi H, Yokoe I, Hirano M, et al. Cardiac magnetic resonance imaging with pharmacological stress perfusion and delayed enhancement in asymptomatic patients with systemic sclerosis. J Rheumatol. 2009; 36(1): 106–112.
  48. Kahan A, Allanore Y. Primary myocardial involvement in systemic sclerosis. Rheumatology. 2006; 45: 14–17.
  49. Steen V. The heart in systemic sclerosis. Curr Rheumatol Rep. 2004; 6(2): 137–140.
  50. Mavrogeni SI, Schwitter J, Gargani L, et al. Cardiovascular magnetic resonance in systemic sclerosis: "Pearls and pitfalls". Semin Arthritis Rheum. 2017; 47(1): 79–85.

Important: This website uses cookies. More >>

The cookies allow us to identify your computer and find out details about your last visit. They remembering whether you've visited the site before, so that you remain logged in - or to help us work out how many new website visitors we get each month. Most internet browsers accept cookies automatically, but you can change the settings of your browser to erase cookies or prevent automatic acceptance if you prefer.

Wydawcą serwisu jest  "Via Medica sp. z o.o." sp.k., ul. Świętokrzyska 73, 80–180 Gdańsk

tel.:+48 58 320 94 94, faks:+48 58 320 94 60, e-mail:  viamedica@viamedica.pl