Generic placeholder image

Current Rheumatology Reviews

Editor-in-Chief

ISSN (Print): 1573-3971
ISSN (Online): 1875-6360

Mini-Review Article

Right Ventricle and Autoimmune Diseases

Author(s): Luis Francisco Valdés Corona* and Tatiana Sofía Rodríguez Reyna

Volume 20, Issue 2, 2024

Published on: 09 October, 2023

Page: [127 - 132] Pages: 6

DOI: 10.2174/0115733971262676230920102922

Price: $65

conference banner
Abstract

Autoimmune diseases can express pathologies in specific organs (e.g. thyroid, pancreas, skin) or generate systemic pathologies (generalized lupus erythematosus, rheumatoid arthritis, systemic sclerosis), the latter usually present systemic inflammatory phenomena.

Some studies have reported alterations in right ventricular contractility in patients with rheumatoid arthritis, systemic lupus erythematosus, systemic sclerosis, and juvenile idiopathic arthritis, which may contribute to the known outcome of increased cardiovascular risk. However, there is not much information available on the causes that generate these alterations, the most likely being small vessel damage and fibrosis due to subclinical inflammation.1-5 In this sense, the disease in which the alterations of the right ventricle have been more studied is systemic sclerosis, specifically at the changes induced due to pulmonary arterial hypertension, this being one of the main causes of death in this group of patients after the significant decrease in mortality associated with the sclerodermic renal crisis with the treatment of angiotensin-converting enzyme inhibitors.

In this review, we will focus on explaining the structural and functional changes that occur in the right ventricle of patients with systemic sclerosis, from early alterations to late complications. In this context, it is necessary to distinguish between right heart alterations that occur in patients with systemic sclerosis and pulmonary arterial hypertension and those that occur without pulmonary arterial hypertension and that can be attributed to other causes such as microvascular damage or myocardial fibrosis.

Keywords: Right ventricle, autoimmune rheumatic disease, pulmonary hypertension, systemic sclerosis, systemic lupus erythematosus, pulmonary arterial hypertension.

Graphical Abstract
[1]
Vizzardi E, Cavazzana I, Bazzani C, et al. Echocardiographic evaluation of asymptomatic patients affected by rheumatoid arthritis. J Investig Med 2012; 60(8): 1204-8.
[http://dx.doi.org/10.2310/JIM.0b013e3182746a83] [PMID: 23111649]
[2]
Buonauro A, Sorrentino R, Esposito R, et al. Three-dimensional echocardiographic evaluation of the right ventricle in patients with uncomplicated systemic lupus erythematosus. Lupus 2019; 28(4): 538-44.
[http://dx.doi.org/10.1177/0961203319833786] [PMID: 30885082]
[3]
Abul MH, Erguven M, Ozben B, Ayhan YI. The forgotten chamber: Right-ventricular functions in juvenile idiopathic arthritis. Pediatr Cardiol 2012; 33(5): 739-43.
[http://dx.doi.org/10.1007/s00246-012-0203-1] [PMID: 22349671]
[4]
Di Minno MND, Forte F, Tufano A, et al. Speckle tracking echocardiography in patients with systemic lupus erythematosus: A meta-analysis. Eur J Intern Med 2020; 73: 16-22.
[http://dx.doi.org/10.1016/j.ejim.2019.12.033] [PMID: 31911023]
[5]
Azpiri-Lopez JR, Galarza-Delgado DA, Colunga-Pedraza IJ, et al. Echocardiographic evaluation of pulmonary hypertension, right ventricular function, and right ventricular-pulmonary arterial coupling in patients with rheumatoid arthritis. Clin Rheumatol 2021; 40(7): 2651-6.
[http://dx.doi.org/10.1007/s10067-020-05544-z] [PMID: 33443606]
[6]
Rodríguez-Reyna TS, Morelos-Guzman M, Hernández-Reyes P, et al. Assessment of myocardial fibrosis and microvascular damage in systemic sclerosis by magnetic resonance imaging and coronary angiotomography. Rheumatology 2015; 54(4): 647-54.
[http://dx.doi.org/10.1093/rheumatology/keu350] [PMID: 25239881]
[7]
Hsu S, Houston BA, Tampakakis E, et al. Right ventricular functional reserve in pulmonary arterial hypertension. Circulation 2016; 133(24): 2413-22.
[http://dx.doi.org/10.1161/CIRCULATIONAHA.116.022082] [PMID: 27169739]
[8]
Bissell LA, Anderson M, Burgess M, et al. Consensus best practice pathway of the UK Systemic Sclerosis Study group: Management of cardiac disease in systemic sclerosis. Rheumatology 2017; 56(6): 912-21.
[http://dx.doi.org/10.1093/rheumatology/kew488] [PMID: 28160468]
[9]
Meune C, Vignaux O, Kahan A, Allanore Y. Heart involvement in systemic sclerosis: Evolving concept and diagnostic methodologies. Arch Cardiovasc Dis 2010; 103(1): 46-52.
[http://dx.doi.org/10.1016/j.acvd.2009.06.009] [PMID: 20142120]
[10]
Kahan A, Allanore Y. Primary myocardial involvement in systemic sclerosis. Rheumatology 2006; 45 (S4): iv14-7.
[http://dx.doi.org/10.1093/rheumatology/kel312] [PMID: 16980717]
[11]
Hachulla A-L, Launay D, Gaxotte V, et al. Cardiac magnetic resonance imaging in systemic sclerosis: A cross-sectional observational study of 52 patients. Ann Rheum Dis 2009; 68(12): 1878-84.
[http://dx.doi.org/10.1136/ard.2008.095836] [PMID: 19054830]
[12]
Hassoun PM. The right ventricle in scleroderma (2013 Grover Conference Series). Pulm Circ 2015; 5(1): 3-14.
[http://dx.doi.org/10.1086/679607] [PMID: 25992267]
[13]
Argula RG, Karwa A, Lauer A, et al. Differences in right ventricular functional changes during treatment between systemic sclerosis-associated pulmonary arterial hypertension and idiopathic pulmonary arterial hypertension. Ann Am Thorac Soc 2017; 14(5): 682-9.
[http://dx.doi.org/10.1513/AnnalsATS.201608-655OC] [PMID: 28282243]
[14]
Kelemen BW, Mathai SC, Tedford RJ, et al. Right ventricular remodeling in idiopathic and scleroderma-associated pulmonary arterial hypertension: two distinct phenotypes. Pulm Circ 2015; 5(2): 327-34.
[http://dx.doi.org/10.1086/680356] [PMID: 26064458]
[15]
Denton CP, Wells AU, Coghlan JG. Major lung complications of systemic sclerosis. Nat Rev Rheumatol 2018.
[16]
Papagoras C, Achenbach K, Tsifetaki N, Tsiouris S, Fotopoulos A, Drosos AA. Heart involvement in systemic sclerosis: A combined echocardiographic and scintigraphic study. Clin Rheumatol 2014; 33(8): 1105-11.
[http://dx.doi.org/10.1007/s10067-014-2666-3] [PMID: 24847773]
[17]
Pigatto E, Peluso D, Zanatta E, et al. Evaluation of right ventricular function performed by 3d-echocardiography in scleroderma patients. Reumatismo 2015; 66(4): 259-63.
[http://dx.doi.org/10.4081/reumatismo.2014.773] [PMID: 25829185]
[18]
Galiè N, Humbert M, Vachiery JL, et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J 2016; 37(1): 67-119.
[http://dx.doi.org/10.1093/eurheartj/ehv317] [PMID: 26320113]
[19]
Steen VD, Follansbee WP, Conte CG, Medsger TA Jr. Thallium perfusion defects predict subsequent cardiac dysfunction in patients with systemic sclerosis. Arthritis Rheum 1996; 39(4): 677-81.
[http://dx.doi.org/10.1002/art.1780390421] [PMID: 8630120]
[20]
Torbicki A. Definition of pulmonary hypertension challenged? Nat Rev Cardiol 2016; 13(5): 250-1.
[http://dx.doi.org/10.1038/nrcardio.2016.44] [PMID: 27009424]
[21]
Weatherald J, Montani D, Jevnikar M, Jaïs X, Savale L, Humbert M. Screening for pulmonary arterial hypertension in systemic sclerosis. Eur Respir Rev 2019; 28(153): 190023.
[http://dx.doi.org/10.1183/16000617.0023-2019] [PMID: 31366460]
[22]
Steen V, Medsger TA Jr. Predictors of isolated pulmonary hypertension in patients with systemic sclerosis and limited cutaneous involvement. Arthritis Rheum 2003; 48(2): 516-22.
[http://dx.doi.org/10.1002/art.10775] [PMID: 12571862]
[23]
Hsu VM, Chung L, Hummers LK, et al. Development of pulmonary hypertension in a high-risk population with systemic sclerosis in the pulmonary hypertension assessment and recognition of outcomes in scleroderma (PHAROS) cohort study. Semin Arthritis Rheum 2014; 44(1): 55-62.
[http://dx.doi.org/10.1016/j.semarthrit.2014.03.002] [PMID: 24709277]
[24]
Rodriguez-Reyna TS, Hinojosa-Azaola A, Martinez-Reyes C, et al. Distinctive autoantibody profile in Mexican Mestizo systemic sclerosis patients. Autoimmunity 2011; 44(7): 576-84.
[http://dx.doi.org/10.3109/08916934.2011.592886] [PMID: 21875377]
[25]
Hsu VM, Chung L, Hummers LK, Sha A, Simms R. Risk factors for mortality and cardiopulmonary hospitalization in systemic sclerosis patients at risk for pulmonary hypertension, in the PHAROS registry. J Rheumatol 2018; 46(2): 176-83.
[26]
Cucuruzac R, Muntean I, Benedek I, et al. Right ventricle remodeling and function in scleroderma patients. BioMed Res Int 2018; 2018: 1-9.
[http://dx.doi.org/10.1155/2018/4528148] [PMID: 29750156]
[27]
Launay D, Sobanski V, Hachulla E, Humbert M. Pulmonary hypertension in systemic sclerosis: Different phenotypes. Eur Respir Rev 2017; 26(145): 170056.
[http://dx.doi.org/10.1183/16000617.0056-2017] [PMID: 28954767]
[28]
Avouac J, Airò P, Meune C, et al. Prevalence of pulmonary hypertension in systemic sclerosis in European Caucasians and metaanalysis of 5 studies. J Rheumatol 2010; 37(11): 2290-8.
[http://dx.doi.org/10.3899/jrheum.100245] [PMID: 20810505]
[29]
Khanna D, Gladue H, Channick R, et al. Recommendations for screening and detection of connective tissue disease-associated pulmonary arterial hypertension. Arthritis Rheum 2013; 65(12): 3194-201.
[http://dx.doi.org/10.1002/art.38172] [PMID: 24022584]
[30]
Allanore Y, Borderie D, Avouac J, et al. High N-terminal pro–brain natriuretic peptide levels and low diffusing capacity for carbon monoxide as independent predictors of the occurrence of precapillary pulmonary arterial hypertension in patients with systemic sclerosis. Arthritis Rheum 2008; 58(1): 284-91.
[http://dx.doi.org/10.1002/art.23187] [PMID: 18163505]
[31]
Chung L, Domsic RT, Lingala B, et al. Survival and predictors of mortality in systemic sclerosis-associated pulmonary arterial hypertension: Outcomes from the pulmonary hypertension assessment and recognition of outcomes in scleroderma registry. Arthritis Care Res 2014; 66(3): 489-95.
[http://dx.doi.org/10.1002/acr.22121] [PMID: 23983198]
[32]
Coghlan JG, Denton CP, Grünig E, et al. Evidence-based detection of pulmonary arterial hypertension in systemic sclerosis: The DETECT study. Ann Rheum Dis 2014; 73(7): 1340-9.
[http://dx.doi.org/10.1136/annrheumdis-2013-203301] [PMID: 23687283]
[33]
Rudski LG, Gargani L, Armstrong WF, et al. Stressing the cardiopulmonary vascular system: The role of echocardiography. J Am Soc Echocardiogr 2018; 31(5): 527-550.e11.
[http://dx.doi.org/10.1016/j.echo.2018.01.002] [PMID: 29573927]
[34]
Suzuki K, Izumo M, Kamijima R, et al. Influence of pulmonary vascular reserve on exercise-induced pulmonary hypertension in patients with systemic sclerosis. Echocardiography 2015; 32(3): 428-35.
[http://dx.doi.org/10.1111/echo.12690] [PMID: 25104236]
[35]
Chia EM, Lau EMT, Xuan W, Celermajer DS, Thomas L. Exercise testing can unmask right ventricular dysfunction in systemic sclerosis patients with normal resting pulmonary artery pressure. Int J Cardiol 2016; 204: 179-86.
[http://dx.doi.org/10.1016/j.ijcard.2015.11.186] [PMID: 26681539]
[36]
Codullo V, Caporali R, Cuomo G, et al. Stress Doppler echocardiography in systemic sclerosis: Evidence for a role in the prediction of pulmonary hypertension. Arthritis Rheum 2013; 65(9): 2403-11.
[http://dx.doi.org/10.1002/art.38043] [PMID: 23754201]
[37]
D’Alto M, Pavelescu A, Argiento P, et al. Echocardiographic assessment of right ventricular contractile reserve in healthy subjects. Echocardiography 2017; 34(1): 61-8.
[http://dx.doi.org/10.1111/echo.13396] [PMID: 27696506]
[38]
Quinn KA, Wappel SR, Kuru T, Steen VD. Exercise echocardiography predicts future development of pulmonary hypertension in a high-risk cohort of patients with systemic sclerosis. J Rheumatol 2020; 47(5): 708-13.
[http://dx.doi.org/10.3899/jrheum.190226] [PMID: 31732551]
[39]
Valdés L, Orihuela-Sandoval C, Muñoz-Hernández L, et al. Should we perform exercise echocardiogram as a screening test for pulmonary arterial hypertension (PAH) for all systemic sclerosis (SSc) patients?. Arthritis Rheumatol 2018; 70(S10)

Rights & Permissions Print Cite
© 2024 Bentham Science Publishers | Privacy Policy