Global Lung Initiative equations for pulmonary hypertension screening in systemic sclerosis

What is it about?

Systemic sclerosis (SSc) and pulmonary hypertension (PH) are both rare diseases. Nevertheless, PH is a harmful complication in about 10% of SSc cases. It is of critical importance to detect PH as soon as possible in patients with SSc because early treatment improves prognosis. An easy-to-perform pulmonary function test, transfer factor of the lung for carbon monoxide, or TLCO, is recognized as a sensitive and specific test for screening for PH in SSc patients when TLCO is expressed as a percentage of predicted value (% pred). Predicted values depend on the reference equations used for their calculation. Universal reference equations for TLCO have recently been established. We aimed to determine the optimal thresholds of TLCO for identification of PH in patients with SSc. We found that a cut-off of 70% predicted (or a z-score of -2.00) in SSc subjects without interstitial lung disease (ILD), and of 50% predicted (or a z-score of -3.50) in SSc patients with ILD are optimal.

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