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Right Ventricular Function in Systemic Autoimmune Diseases

  • Ioan Tiberiu Nanea
  • Gabriela Silvia Gheorghe
Chapter

Abstract

Systemic autoimmune diseases (SAD) can be defined as inflammatory diseases with an immune mechanism, of unknown etiology, involving at least 2 organs or systems. This category includes collagen-vascular diseases, vasculitides, granulomatous diseases, while excluding systemic inflammatory diseases with known causes. Cardiovascular changes are among the most frequent causes of morbi-mortality in patients with SAD by many mechanisms, including sustained systemic inflammation. Chronic right ventricular (RV) involvement in SAD may be due to long-term left ventricular (LV) decompensation, inflammation and myocardial fibrosis generated by the disease itself, pulmonary parenchymal changes and/or pulmonary hypertension (PAH), chronic repetitive pulmonary embolism. These differ in type, prevalence, intensity between the various forms of SAD. Systemic sclerosis (SSc) is the most frequent disease that causes lung and cardiac involvement. SS generates PAH by multiple mechanisms mainly in the cutaneous form, although it induces myocardial perfusion defects especially in the diffuse form. The RV involvement occurs earlier in SSc than in other forms of PAH and the prognosis is more severe. However, the new cardiac imaging techniques prove the occurrence of the systolic and diastolic dysfunction in right and left ventricles in cases without established PAH and clinical heart involvement. Systemic erythematosus lupus (SLE), mixed connective-tissue disease (MCTD), Sjogren syndrome (SjS), rheumatoid arthritis (RA) determine less often PAH and RV involvement. There are also other mechanisms for the cardiac involvement in SAD, like coronaritis, accelerated atherosclerosis or pericarditis.

Keywords

Systemic sclerosis Systemic erythematosus lupus Rheumatoid arthritis Pulmonary hypertension Right ventricular function TAPSE TEI index Longitudinal strain Longitudinal strain rate 

References

  1. 1.
    Knockaert DC. Cardiac involvement in systemic inflammatory diseases. Eur Heart J. 2007;28(15):1797–804.CrossRefPubMedGoogle Scholar
  2. 2.
    Luqmani RA, Suppiah R, Grayson PC, Merkel PA, Watts R. Nomenclature and classification of vasculitis—update on the ACR/EULAR Diagnosis and Classification of Vasculitis Study (DCVAS). Clin Exp Immunol. 2011;164(Suppl 1):11–3.CrossRefPubMedPubMedCentralGoogle Scholar
  3. 3.
    Lazzerini PE, Capecchi PL, Laghi-Pasini F. Systemic inflammation and arrhythmic risc: lessons from rheumatoid arthritis. Eur Heart J. 2017;38(22):1717–27.PubMedGoogle Scholar
  4. 4.
    Dinarello CA. Immunological and inflammatory functions of the interleukin-1 family. Annu Rev Immunol. 2009;27:519–50.CrossRefPubMedGoogle Scholar
  5. 5.
    Watts TH. TNF/TNFR family members in costimulation of T cell responses. Annu Rev Immunol. 2005;23:23–68.CrossRefPubMedGoogle Scholar
  6. 6.
    Shoenfeld Y, Gerli R, Doria A, Matsuura E, Cerinic MM, Ronda N, Jara LJ, et al. Accelerated atherosclerosis in autoimmune rheumatic diseases. Circulation. 2005;112(22):3337–47.CrossRefPubMedGoogle Scholar
  7. 7.
    Cosgrove GP, Schwarz MI. Pulmonary manifestations of the collagen vascular diseases. https://thoracickey.com/pulmonarymanifestationsofthecollagenvasculardiseases/.
  8. 8.
    Capobianco J, Grimberg A, Thompson BM, Antunes VB, Jasinowodolinski D, Meirelles GSP. Thoracic manifestations of collagen vascular diseases. Radio Graphics. 2012;32(1):33–50.Google Scholar
  9. 9.
    Cosgrove GP, Schwarz MI. Pulmonary manifestations of the collagen vascular diseases, chapter 60, p 902–918. In: Fishman’ s pulmonary diseases and disorders. 15th ed. New York: McGraw-Hill; 2015.Google Scholar
  10. 10.
    Noordegraaf AV, Westerhof BE, Westerhof N. The relationship between the right ventricle and its load in pulmonary hypertension. J Am Coll Cardiol. 2017;69(2):236–43.CrossRefGoogle Scholar
  11. 11.
    Naejije R, Brimioulle S, Dewachter L. Biomechanics of the right ventricle in health and disease (2013 Grover conference series). Pulm Circ. 2014;4(3):395–406.CrossRefGoogle Scholar
  12. 12.
    Vonk-Noordegraaf A, Haddad F, Chin KM, Forfia PR, Kawut SM, Lumens J, et al. Right heart adaptation to pulmonary arterial hypertension: physiology and pathobiology. J Am Coll Cardiol. 2013;62(suppl 25):D22–33.CrossRefPubMedGoogle Scholar
  13. 13.
    Condliffe R, Howard LS. Connective tissue disease-associated pulmonary arterial hypertension. F1000Prime Rep. 2015;7:06.  https://doi.org/10.12703/P7-06.CrossRefPubMedPubMedCentralGoogle Scholar
  14. 14.
    D’Andrea A, Stisi S, Bellissimo S, Vigorito F, di Uccio FS, Tozzi N, et al. Early impairment of myocardial function in systemic sclerosis: non-invasive assessment by Doppler myocardial and strain rate imaging. Eur J Echocardiography. 2005;6(6):407–18.CrossRefGoogle Scholar
  15. 15.
    Hassoun PM. The right ventricle in scleroderma (2013 Grover conference series). Pulm Circ. 2015;5(1):3–14.CrossRefPubMedPubMedCentralGoogle Scholar
  16. 16.
    Overbeek MJ, Mouchaers KTB, Niessen HM, Hadi AM, Kupreishvili K, Boonstra A, et al. Characteristics of interstitial fibrosis and inflammatory cell infiltration in right ventricles of systemic sclerosis-associated pulmonary arterial hypertension. Int J Rheumatol. 2010:604615.  https://doi.org/10.1155/2010/604615.
  17. 17.
    Vonk MC, Sande MH, van den Hoogen FHJ, van Riel PLCM, Verheugt FWA, van Dijk APJ. Right ventricle Tei-index: a tool to increase the accuracy of non-invasive detection of pulmonary arterial hypertension in connective tissue diseases. Eur J Echocardiogr. 2007;8(5):317–21.CrossRefPubMedGoogle Scholar
  18. 18.
    Kepez A, Akdogan A, Sade LE, Deniz A, Kalyoncu U, Karadag O, Hayran M, Aytemir K, Ertenli I, Kiraz S, Calguneri M, Kabakci G, Tokgozoglu L. Detection of subclinical cardiac involvement in systemic sclerosis by echocardiographic strain imaging. Echocardiography. 2008;25(2):191–7.CrossRefPubMedGoogle Scholar
  19. 19.
    Pigatto E, Peluso D, Zanatta E, Polito P, Miatton P, Bourji K, et al. Evaluation of right ventricular function performed by 3d-echocardiography in scleroderma patients. Reumatismo. 2014;66(4):259–63.CrossRefGoogle Scholar
  20. 20.
    Loureiro MJ, Cotrim C, Simoes O, Cordeiro A, Santos MJ, Silva C, et al. Exercise Doppler echocardiography in the detection of pulmonary arterial hypertension in collagen vascular disease, P 1139, European congress of echocardiography. Eur J of Echocardiogr. 2005;6(suppl 1):S184–5.Google Scholar
  21. 21.
    Giunta A, Tirri E, Maione S, Cangianiello S, Mele A, DeLuca A, et al. Right ventricular diastolic abnormalities in systemic sclerosis. Relation to left ventricular involvement and pulmonary hypertension. Ann Rheum Dis. 2000;59(2):94–8.CrossRefPubMedPubMedCentralGoogle Scholar
  22. 22.
    Rosato E, Maione S, Vitarelli A, Giunta A, Fontanella L, de Horatio LT, et al. Regional diastolic function by tissue Doppler echocardiography in systemic sclerosis: correlation with clinical variables. Rheumatol Int. 2009;29(8):913–9.CrossRefPubMedGoogle Scholar
  23. 23.
    Nakajima K, Taki J, Kawano M, Higuchi T, Sato S, Nishijima C, et al. Diastolic dysfunction in patients with systemic sclerosis detected by gated myocardial perfusion SPECT: an early sign of cardiac involvement. J Nucl Med. 2001;42(2):183–8.PubMedGoogle Scholar
  24. 24.
    Lynch DA. Lung disease related to collagen vascular disease. J Thorac Imaging. 2009;24(4):299–309.CrossRefPubMedGoogle Scholar
  25. 25.
    Lang R, Goldstein SA, Kronzon I, Khandheria BK, Mor-Avi V. ASE’s comprehensive echocardiography. 2nd ed. Amsterdam: Elsevier; 2016.Google Scholar
  26. 26.
    Poorzand H, Mirfeizi SZ, Javanbakht A, Alimi H. Comparison of echocardiographic variables between systemic lupus erythematosus patients and a control group. Arch Cardiovasc Imaging. 2015;3(2):e30009.  https://doi.org/10.5812/acvi.30009.CrossRefGoogle Scholar
  27. 27.
    Elnady BM, Abdelghafar ASM, Khalik ESA, Algethami MM, Basiony AS, Al-otaibi MDA, et al. The implication of tissue Doppler echocardiography and cardiopulmonary exercise in early detection of cardiac dysfunction in systemic lupus erythematosus patients. Eur J Rheumatol. 2016;3(3):109–17.  https://doi.org/10.5152/eurjrheum.2016.16002.CrossRefPubMedPubMedCentralGoogle Scholar
  28. 28.
    Tektonidou MG, Ioannidis JP, Moyssakis I, Boki KA, Vassiliou V, Vlachoyiannopoulos PG, et al. Right ventricular diastolic dysfunction in patients with anticardiolipin antibodies and antiphospholipid syndrome. Ann Rheum Dis. 2001;60(1):43–8.CrossRefPubMedPubMedCentralGoogle Scholar
  29. 29.
    Leal GN, Silva KF, Franca CM, Lianza AC, Andrade JL, Campos LM, et al. Subclinical right ventricle systolic dysfunction in childhood-onset systemic lupus erythematosus: insights from two-dimensional speckle-tracking echocardiography. Lupus. 2015;24(6):613–20.CrossRefPubMedGoogle Scholar
  30. 30.
    Vég J, Hegedűs I, Szegedi G, Zeher M, Bodolay E. Diastolic function of the heart in mixed connective tissue disease. Clin Rheumatol. 2007;26(2):176–81.CrossRefGoogle Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  • Ioan Tiberiu Nanea
    • 1
    • 2
  • Gabriela Silvia Gheorghe
    • 1
    • 2
  1. 1.University of Medicine and Pharmacy “Carol Davila”BucharestRomania
  2. 2.“Prof Dr Th Burghele” University HospitalBucharestRomania

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