Screening for pulmonary arterial hypertension in systemic sclerosis

Eur Respir Rev. 2009 Sep;18(113):162-9. doi: 10.1183/09059180.00003209.


The onset and progression of pulmonary arterial hypertension (PAH) in patients with systemic sclerosis (SSc) can be particularly aggressive; however, effective treatments are available. Therefore, early identification of patients with suspected PAH, confirmation of diagnosis, and intervention is essential. PAH may be challenging to diagnose in its earliest stages, particularly in populations that have multiple causes of breathlessness, and, therefore, screening is required. The optimal screening tools and methodology are, as yet, unknown, and this is confounded by a lack of consensus over which patients to screen. Current practice favours annual screening of all SSc patients using Doppler echocardiography to detect elevated right heart pressures. This will typically identify most patients with the various forms of pulmonary hypertension found in SSc. The optimum thresholds for Doppler echocardiography are still subject to investigation, especially for patients with mild pulmonary hypertension, and this technique may, therefore, yield a significant number of false-positives and a currently unknown number of false-negatives. Confirmatory right heart catheterisation remains necessary in all suspected cases. Further research is needed to identify the optimal tools and the screening approach with greatest specificity and selectivity.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Echocardiography, Doppler*
  • Familial Primary Pulmonary Hypertension
  • Humans
  • Hypertension, Pulmonary / complications
  • Hypertension, Pulmonary / diagnostic imaging
  • Mass Screening / methods*
  • Scleroderma, Systemic / complications*
  • Sensitivity and Specificity