Figure 1. Suggested screening protocol for the detection of PAH in SSc patients4 - click to enlarge
Figure adapted from: The three-year incidence of pulmonary arterial hypertension associated with systemic sclerosis in a multicenter nationwide longitudinal study in France. Hachulla E, de Groote P, Gressin V, et al. Copyright © 2009, John Wiley and Sons, Inc. Reproduced with permission of John Wiley and Sons, Inc.
The diagnosis of PAH in patients with SSc can be particularly challenging, especially in its early stages. SSc affects a number of organs, including the lungs, and can be associated with symptoms such as fatigue and dyspnoea, which are also symptoms of PAH. As a result, detection of PAH in SSc is often delayed and patients are only diagnosed when they have advanced disease with severe clinical and haemodynamic impairment.1
However, given the known high incidence of PAH in SSc, echocardiography screening is recommended in all symptomatic patients and screening by echocardiography may also be considered in all SSc patients according to the treatment guidelines.2,3 Recent studies have also shown that screening for PAH in asymptomatic SSc patients is associated with improved outcomes.1
Figure 1 shows a suggested screening algorithm.4 In this algorithm, it is recommended that patients who have SSc without severe pulmonary function abnormalities or severe cardiac disease are regularly assessed using Doppler echocardiography. The parameter assessed is peak velocity of tricuspid regurgitation (VTR); this measure can be used to calculate pulmonary arterial pressure (PAP), which is elevated in patients with PAH (see section: How is PAH diagnosed?). Patients with low VTR (<2.8 m/s) do not have PAH, whereas a high (>3.0 m/s) VTR or moderate VTR (2.8-3.0 m/s) together with dyspnoea, raises the suspicion of PAH. To confirm the diagnosis, patients must then undergo right heart catheterisation (RHC) (see section: How is PAH diagnosed?).
- Humbert M, Yaici A, de Groote P, et al. Screening for pulmonary arterial hypertension in patients with systemic sclerosis: clinical characteristics at diagnosis and long-term survival. Arthritis Rheum 2011;63:3522–30.
- Galiè N, Hoeper MM, Humbert M, et al. Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J 2009;30:2493–537.
- McLaughlin VV, Archer SL, Badesch DB, et al. ACCF/AHA. ACCF/AHA 2009 expert consensus document on pulmonary hypertension: a report of the American College of Cardiology Foundation Task Force on Expert Consensus Documents and the American Heart Association: developed in collaboration with the American College of Chest Physicians, American Thoracic Society, Inc., and the Pulmonary Hypertension Association. Circulation. 2009;119:2250–94.
- Hachulla E, de Groote P, Gressin V, et al. The three-year incidence of pulmonary arterial hypertension associated with systemic sclerosis in a multicenter nationwide longitudinal study in France. Arthritis Rheum 2009;60:1831–9.