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What is PAH?

How is PAH-SSc treated?

PAH-SSc is associated with poor outcome if untreated. Data suggest that available therapies may improve quality of life and exercise capacity, and slow disease progression.1-5

Overall, treatment of patients with PAH-SSc should follow the same treatment algorithm as IPAH patients (see section: How is PAH treated).6 There are, however, some differences. For example, long-term favourable response to calcium channel blocker (CCB) treatment in vasoreactive patients with PAH-SSc is seen even less often than in idiopathic PAH (IPAH), and the risk-to-benefit ratio of oral anticoagulation is not well understood.6,7

Patients should also be followed and reassessed regularly in the same way as other PAH patients. Similar parameters can be used with some additional considerations. The six-minute walk test (6MWT) used to evaluate exercise capacity is an important measure in the routine evaluation of patients with PAH (see section: Assessing the severity of PAH). However, the 6MWT has only been validated in patients with IPAH. Patients with PAH-SSc have a number of complications, such as other cardiopulmonary disorders, musculoskeletal pain, fatigue, arthritis/tendonitis, and muscle weakness and contractures, which can interfere with their ability to perform the test and therefore affect results.8

Treatment of patients with PAH-SSc is also complicated by the need to manage other problems associated with SSc, such as skin, renal, and gastrointestinal complications.9 As with other forms of PAH, multidisciplinary care is important, and patients should therefore be referred to an expert centre for PAH diagnosis and management.



  1. Denton CP, Humbert M, Rubin L, Black CM. 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;65:1336–40.
  2. Launay D, Sitbon O, Le Pavec J, et al. Long-term outcome of systemic sclerosis-associated pulmonary arterial hypertension treated with bosentan as first-line monotherapy followed or not by the addition of prostanoids or sildenafil. Rheumatology (Oxford) 2010;49:490–500.
  3. Badesch DB, Hill NS, Burgess G, et al. Sildenafil for pulmonary arterial hypertension associated with connective tissue disease. J Rheumatol 2007;34:2417–22.
  4. Badesch DB, McGoon MD, Barst RJ, et al. Long-term survival among patients with scleroderma-associated pulmonary arterial hypertension treated with intravenous epoprostenol. J Rheumatol 2009;36:2244–9.
  5. Oudiz RJ, Schilz RJ, Barst RJ, et al. Treprostinil, a prostacyclin analogue, in pulmonary arterial hypertension associated with connective tissue disease. Chest 2004;126:420–7.
  6. Galiè N, Hoeper MM, Humbert M, et al. Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J 2009;30:2493–537.
  7. Badesch DB, Abman SH, Ahearn GS, et al. Medical therapy for pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. Chest 2004;126(1 Suppl):35S–62S.
  8. Garin MC, Highland KB, Silver RM, Strange C. Limitations to the six-minute walk test in interstitial lung disease and pulmonary hypertension in scleroderma. J Rheumatol 2009;36:330–6.
  9. Kowal-Bielecka O, Landewé R, Avouac J, et al. EULAR recommendations for the treatment of systemic sclerosis: a report from the EULAR Scleroderma Trials and Research group (EUSTAR). Ann Rheum Dis 2009;68:620–8.