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What is PAH?

PAH in patients with systemic sclerosis (SSc)

Around 15% of all cases of pulmonary arterial hypertension (PAH) in a recent French PAH registry1 and 25% in a US PAH registry2 were associated with connective tissue disease, and particularly with SSc (also known as scleroderma) (see section: Classification of PH).

The prevalence of PAH in patients with connective tissue disease has been well established only for patients with SSc.3 In recent studies, the prevalence of right heart catheterisation-confirmed PAH in patients with SSc was between 7% and 12%.4,5 Patients with SSc who develop PAH have poorer prognosis than those who do not.6,7 For example, a study of patients from the large Pittsburgh Scleroderma Databank aimed to compare survival in 106 patients with SSc and a diagnosis of PAH with 106 age- and disease-matched controls.7 Patients with SSc and PAH had a 2-year cumulative survival of 50% and a 5-year cumulative survival of 10% following diagnosis of PAH, compared with 88% and 80%, respectively, for the control group measured from a matching time.7

PAH has become one of the leading causes of mortality in SSc patients and accounts for more than 25% of all SSc-related deaths.8

Given the significant incidence of PAH in patients with SSc and the high mortality associated with this complication if untreated, there is a clear need for early detection and timely treatment before patients show marked clinical and haemodynamic deterioration.4

If a patient with SSc shows symptoms that may be attributable to PAH (see section: What are the symptoms of PAH?) or if you suspect PAH for any reason, consider referring the patient to a centre with expertise in PAH for assessment.



  1. 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;173:1023–30.
  2. Badesch DB, Raskob GE, Elliott CG, et al. Pulmonary arterial hypertension: baseline characteristics from the REVEAL Registry. Chest 2010;137:376–87.
  3. Simonneau G, Robbins I, Beghetti M, et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol 2009;54:S43–S54.
  4. 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;52:3792–800.
  5. 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;62:1088–93.
  6. Hachulla E, Carpentier P, Gressin V, et al. Risk factors for death and the 3-year survival of patients with systemic sclerosis: the French ItinérAIR-Sclérodermie study. Rheumatology (Oxford). 2009;48:304–8.
  7. Steen V, Medsger TA Jr. Predictors of isolated pulmonary hypertension in patients with systemic sclerosis and limited cutaneous involvement. Arthritis Rheum. 2003;48:516–22.
  8. Steen VD, Medsger TA. Changes in causes of death in systemic sclerosis, 1972-2002. Ann Rheum Dis 2007;66:940–4.