Abstract
Systemic sclerosis associated pulmonary arterial hypertension (SSc-PAH) is an important cause of morbidity and mortality in patients with systemic sclerosis (SSc). Although studies based on echocardiography-derived pulmonary artery pressures have resulted in estimates of the prevalence of pulmonary arterial hypertension (PAH) in patients with SSc as high as 35%, more recent studies, using catheter-based diagnoses, have produced estimates of between 7.8 and 12%.1–3 Historically, SSc-PAH has had a poor outlook with rapid clinical deterioration and a 3-year survival of 30%.4 This was worse than in idiopathic pulmonary arterial hypertension (iPAH) wherein median survival prior to disease-modifying therapy was 2.8 years.5
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
References
Battle R, Davitt M, Cooper SM, et al. Prevalence of pulmonary hypertension in limited and diffuse scleroderma. Chest. 1996;110:1515-1519.
Hachulla E, Gressin V, Guillevin L, et al. Early detection of pulmonary arterial hypertension in systemic sclerosis: a French nationwide prospective multicenter study. Arthritis Rheum. 2005;52:3792-3800.
Mukerjee D, St George D, Coleiro B, et al. Prevalence and outcome in systemic sclerosis associated pulmonary arterial hypertension: application of a registry approach. Ann Rheum Dis. 2003;62:1088-1093.
Koh ET, Lee P, Gladman DD, et al. Pulmonary hypertension in systemic sclerosis: an analysis of 17 patients. Br J Rheumatol. 1996;35:989-993.
D’Alonzo GE, Barst RJ, Ayres SM, et al. Survival in patients with primary pulmonary hypertension Results from a national prospective registry. Ann Intern Med. 1991;115:343-349.
Higenbottam T, Wheeldon D, Wells F, et al. Long-term treatment of primary pulmonary hypertension with continuous intravenous epoprostenol (prostacyclin). Lancet. 1984;1:1046-1047.
Barst RJ, Rubin LJ, Long WA, et al. A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension. The primary pulmonary hypertension study group. N Engl J Med. 1996;334:296-302.
Giaid A, Yanagisawa M, Langleben D, et al. Expression of endothelin-1 in the lungs of patients with primary pulmonary hypertension. N Engl J Med. 1993;328:1732-1739.
Rubin LJ, Badesch DB, Barst RJ, et al. Bosentan therapy for pulmonary arterial hypertension. N Engl J Med. 2002;346:896-903.
Barst RJ, Langleben D, Frost A, et al. Sitaxsentan therapy for pulmonary arterial hypertension. Am J Respir Crit Care Med. 2004;169:441-447.
Galie N, Ghofrani HA, Torbicki A, et al. Sildenafil citrate therapy for pulmonary arterial hypertension. N Engl J Med. 2005;353:2148-2157.
Highland KB, Strange C, Girgis R, et al. Comparison of sitaxentan and bosentan in pulmonary arterial hypertension associated with connective tissue diseases [abstract]. Ann Rheum Dis. 2006;65:A393.
Humbert M, Barst RJ, Robbins IM, et al. Combination of bosentan with epoprostenol in pulmonary arterial hypertension: BREATHE-2. Eur Respir J. 2004;24:353-359.
Badesch DB, Tapson VF, McGoon MD, et al. Continuous intravenous epoprostenol for pulmonary hypertension due to the scleroderma spectrum of disease. A randomized, controlled trial. Ann Intern Med. 2000;132:425-434.
Humbert M, Sanchez O, Fartoukh M, et al. Short-term and long-term epoprostenol (prostacyclin) therapy in pulmonary hypertension secondary to connective tissue diseases: results of a pilot study. Eur Respir J. 1999;13:1351-1356.
Galie N, Humbert M, Vachiery JL, et al. Effects of beraprost sodium, an oral prostacyclin analogue, in patients with pulmonary arterial hypertension: a randomized, double-blind, placebo-controlled trial. J Am Coll Cardiol. 2002;39:1496-1502.
Barst RJ, McGoon M, McLaughlin V, et al. Beraprost therapy for pulmonary arterial hypertension. J Am Coll Cardiol. 2003;41:2119-2125.
Simonneau G, Barst RJ, Galie N, et al. Continuous subcutaneous infusion of treprostinil, a prostacyclin analogue, in patients with pulmonary arterial hypertension: a double-blind, randomized, placebo-controlled trial. Am J Respir Crit Care Med. 2002;165:800-804.
Oudiz RJ, Schilz RJ, Barst RJ, et al. Treprostinil, a prostacyclin analogue, in pulmonary arterial hypertension associated with connective tissue disease. Chest. 2004;126:420-427.
Launay D, Hachulla E, Hatron PY, et al. Aerosolized iloprost in CREST syndrome related pulmonary hypertension. J Rheumatol. 2001;28:2252-2256.
Badesch D, Burgess G, Parpia T, et al. Sildenafil improves exercise ability and hemodynamics in patients with pulmonary arterial hypertension associated with connective tissue disease. J Rheumatol. 2007;34:2417-2422.
Rich S. The current treatment of pulmonary arterial hypertension: time to redefine success. Chest. 2006;130:1198-1202.
Merkel P, Clements PJ, Reveille JD, et al. Current status of outcome measure development for clinical trials in systemic sclerosis. Report from OMERACT 6. J Rheumatol. 2003;30:1630-1647.
Distler O, Behrens F, Huscher D, et al. Need for improved outcome measures in pulmonary arterial hypertension related to systemic sclerosis. Rheumatology. 2006;45:1455-1457.
Furst D. Measuring outcome in PAH: the gap between the measures that are used and their validity. Ann N Y Acad Sci. 2007;1107:410-416.
Miyamoto S, Nagaya N, Satoh T, et al. Clinical correlates and prognostic significance of six-minute walk test in patients with primary pulmonary hypertension Comparison with cardiopulmonary exercise testing. Am J Respir Crit Care Med. 2000;161:487-492.
Sitbon O, Humbert M, Nunes H, et al. Long-term intravenous epoprostenol infusion in primary pulmonary hypertension: prognostic factors and survival. J Am Coll Cardiol. 2002;40:780-788.
Galie N, Rubin LJ, Hoeper MM, et al. Bosentan improves hemodynamics and delays time to clinical worsening in patients with mildly symptomatic Pulmonary Arterial Hypertension (PAH): results of the EARLY study [abstract]. Eur Heart J. 2007;28:A140.
Condliffe R, Kiely D, Peacock A, et al. Initial results of the United Kingdom audit of pulmonary arterial hypertension associated with connective tissue disease [abstract]. Eur Heart J. 2007;28:1010S.
Williams MH, Das C, Handler CE, et al. Systemic sclerosis associated pulmonary hypertension: improved survival in the current era. Heart. 2006;92:926-932.
Denton CP, Humbert M, Rubin L, et al. Bosentan treatment for pulmonary arterial hypertension related to connective tissue disease: a subgroup analysis of the pivotal clinical trials and their open-label extensions. Ann Rheum Dis. 2006;65:1336-1340.
Kawut SM, Taichman DB, Archer CCL, et al. Hemodynamics and survival in patients with pulmonary arterial hypertension related to systemic sclerosis. Chest. 2003;123:344-350.
McLaughlin VV. Survival in patients with pulmonary arterial hypertension treated with first-line bosentan. Eur J Clin Invest. 2006;36:10-15.
McLaughlin VV, Shillington A, Rich S. Survival in primary pulmonary hypertension: the impact of epoprostenol therapy. Circulation. 2002;106:1477-1482.
Humbert M, Sitbon O, Chaouat A, et al. Pulmonary arterial hypertension in France: results from a national registry. Am J Respir Crit Care Med. 2006;173:1023-1030.
Scully R, Mark E, McNeely W, et al. Case records of the Massachusetts general hospital. N Engl J Med. 1989;320:1333-1340.
Steen V, Conte C, Owens GR, et al. Severe restrictive lung disease in systemic sclerosis. Arthritis Rheum. 1994;37:1283-1289.
Morgan C, Knight C, Lunt M, et al. Predictors of end stage lung disease in a cohort of patients with scleroderma. Ann Rheum Dis. 2003;62:146-150.
Steen V, Medsger TA Jr. Severe organ involvement in systemic sclerosis with diffuse scleroderma. Arthritis Rheum. 2000;43:2437-2444.
Owens GR, Follansbee W. Cardiopulmonary manifestations of systemic sclerosis. Chest. 1987;91:118-127.
Follansbee W, Miller T, Curtiss E, et al. A controlled clinicopathologic study of myocardial fibrosis in systemic sclerosis (scleroderma). J Rheumatol. 1990;17:656-662.
Di Bello V, Ferri C, Giorgi D, et al. Ultrasonic videodensitometric analysis in scleroderma heart disease. Coron Artery Dis. 1999;10:103-110.
Meune C, Allanore Y, Pascal O, et al. Myocardial contractility is affected early in systemic sclerosis: A tissue Doppler echocardiography study. Eur J Echocardiogr. 2005;6:351-357.
Meune C, Allanore Y, Devaux J, et al. High prevalence of right ventricular systolic dysfunction in early systemic sclerosis. J Rheumatol. 2004;31:1941-1945.
Giunta A, Tirri E, Maione S, et al. Right ventricular diastolic abnormalities in systemic sclerosis. Relation to left ventricular involvement and pulmonary hypertension. Ann Rheum Dis. 2000;59:94-98.
Lindqvist P, Caidahl K, Neuman-Andersen G, et al. Disturbed right ventricular diastolic function in patients with systemic sclerosis. Chest. 2005;128:755-763.
Huez S, Roufosse F, Vachiery JL, et al. Isolated right ventricular dysfunction in systemic sclerosis: latent pulmonary hypertension? Eur Respir J. 2007;30:928-936.
Vlachoyiannopoulus P, Dafni U, Pakas I, et al. Systemic scleroderma in Greece: low mortality and strong linkage with HLA-DRB1*1104 allele. Ann Rheum Dis. 2000;59:359-367.
Tuder RM, Zaiman A. Pathology of pulmonary vascular disease. In: Peacock AJ, Rubin LJ, eds. Pulmonary Circulation. 2nd ed. London: Arnold; 2004:25-32.
Kawut SM, Horn E, Berekashvili K, et al. New predictors of outcome in idiopathic pulmonary arterial hypertension. Am J Cardiol. 2005;95:199-203.
Shapiro B, McGoon M, Redfield M. Unexplained pulmonary hypertension in elderly patients. Chest. 2007;131:94-100.
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2009 Springer-Verlag London
About this chapter
Cite this chapter
Condliffe, R. (2009). Disease Progression in Systemic Sclerosis Associated Pulmonary Arterial Hypertension. In: Abraham, D., Clive, H., Dashwood, M., Coghlan, G. (eds) Advances in Vascular Medicine. Springer, London. https://doi.org/10.1007/978-1-84882-637-3_17
Download citation
DOI: https://doi.org/10.1007/978-1-84882-637-3_17
Published:
Publisher Name: Springer, London
Print ISBN: 978-1-84882-636-6
Online ISBN: 978-1-84882-637-3
eBook Packages: MedicineMedicine (R0)