Abstract
Background
Systemic sclerosis (SSc) is an important cause of pulmonary arterial hypertension (PAH), with an estimated prevalence of 7.85–26.7%.
Objective
Our aim was to estimate the prevalence of PAH among patients with SSc in the Czech Republic and to compare haemodynamics in SSc patients diagnosed with PAH through screening with those diagnosed previously, based on symptoms.
Methods
During 2007, SSc patients in the Czech Republic, without significant pulmonary function impairment or cardiac disease, underwent screening for PAH with transthoracic echocardiography. Those with a tricuspid regurgitant (TR) jet gradient suggestive of PAH (>30 mmHg) underwent subsequent right heart catheterisation (RHC) to confirm the diagnosis (mean pulmonary arterial pressure, mPAP, ≥25 mmHg; pulmonary capillary wedge pressure, ≤15 mmHg). Haemodynamics in patients diagnosed with PAH in this way were compared with those in patients diagnosed previously, based on symptoms.
Results
Two hundred and three SSc patients (mean age, 53.8 ± 13 years; 82.3% women) from 26 rheumatology practices were screened. Among these, 17 had a TR jet gradient >30 mmHg and underwent RHC; PAH was confirmed in six patients. These six patients were found to have significantly lower mPAP than nine patients diagnosed previously with PAH, based on symptoms (31.17 ± 5.56 vs. 46.89 ± 9.48 mmHg, p = 0.0014).
Conclusion
Prevalence of PAH in our SSc cohort was 7.08%. SSc patients diagnosed with PAH through screening have less advanced disease in terms of haemodynamics than those with PAH diagnosed previously based on symptoms; their prognosis is therefore likely to be more favourable.
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References
Galiè N, Hoeper MM, Humbert M et al (2009) Guidelines for the diagnosis and treatment of pulmonary hypertension. The task force for the diagnosis and treatment of pulmonary hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS), endorsed by the International Society of Heart and Lung Transplantation (ISHLT). Eur Heart J 30:2493–2537
Steen V, Medsger TA (2003) Predictors of isolated pulmonary hypertension in patients with systemic sclerosis and limited cutaneous involvement. Arthritis Rheum 48:516–522
Steen V (2003) Predictors of end stage lung disease in systemic sclerosis. Ann Rheum Dis 62:97–99
Kawut SM, Taichman DB, Archer-Chicko DB et al (2003) Hemodynamics and survival in patients with pulmonary arterial hypertension related to systemic sclerosis. Chest 123:344–350
Koh ET, Lee P, Gladman DD et al (1996) Pulmonary hypertension in systemic sclerosis: an analysis of 17 patients. Br J Rheumatol 35:989–993
Chang B, Wigley FM, White B et al (2003) Scleroderma patients with combined pulmonary hypertension and interstitial lung disease. J Rheumatol 30:2398–2405
Hesselstrand R, Ekman R, Eskilsson J et al (2005) Screening for pulmonary hypertension in systemic sclerosis: the longitudinal development of tricuspid gradient in 227 consecutive patients, 1992–2001. Rheumatology 44:366–371
Wigley FM, Lima JA, Mayes M et al (2005) The prevalence of undiagnosed pulmonary arterial hypertension in subjects with connective tissue disease at the secondary health care level of community-based rheumatologists (the UNCOVER study). Arthritis Rheum 52:2125–2132
Murata I, Takenaka K, Yoshinoya S et al (1997) Clinical evaluation of pulmonary hypertension in SSc and related disorders. A Doppler echocardiographic study of 135 Japanese patients. Chest 111:36–43
MacGregor AJ, Canavan R, Knight C et al (2001) Pulmonary hypertension in systemic sclerosis: risk factors for progression and consequences for survival. Rheumatology 40:453–459
Mukerjee D, St George D, Coleiro B et al (2003) Prevalence and outcome in systemic sclerosis associated pulmonary arterial hypertension: an application of registry approach. Ann Rheum Dis 62:10888–10893
Hachulla E, Gressin V, Guillevin L et al (2005) Early detection of pulmonary arterial hypertension in systemic sclerosis. A French nationwide prospective multicenter study. Arthritis Rheum 52:3792–3800
Pope JE, Lee P, Baron M et al (2005) Prevalence of elevated pulmonary arterial pressures measured by echocardiography in a multicenter study of patients with systemic sclerosis. J Rheumatol 32:1273–1278
Daniels LB, Krummen DE, Blanchard DG (2004) Echocardiography in pulmonary vascular disease. Cardiol Clin 22:383–399
Denton CP, Cailes JB, Phillips GD et al (1997) Comparison of Doppler echocardiography and right heart catheterization to assess pulmonary hypertension in systemic sclerosis. Br J Rheumatol 36:239–243
Acknowledgements
The authors would like to thank all those who contributed to this study (see attached list), Professor Christopher Denton for his valuable feedback and Elements Communications Ltd. for providing medical writing assistance during the preparation of this manuscript, funded by Actelion Pharmaceuticals Ltd. This study was supported by a grant from the Czech Society of Cardiology.
Disclosures
Pavel Jansa received honoraria, consultancy fees and grants from Actelion Pharmaceuticals, Pfizer, Bayer, United Therapeutics and AOP Orphan Pharmaceuticals. Radim Becvar, David Ambroz, Tomas Palecek, Michal Tomcik, Simona Skacelova, Michael Aschermann and Ales Linhart have no conflict of interest declared.
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Jansa, P., Becvar, R., Ambroz, D. et al. Pulmonary arterial hypertension associated with systemic sclerosis in the Czech Republic. Clin Rheumatol 31, 557–561 (2012). https://doi.org/10.1007/s10067-011-1896-x
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DOI: https://doi.org/10.1007/s10067-011-1896-x