Objective: To define the risk factors associated with a relatively rapid increase in estimated pulmonary arterial systolic pressure (PASP) in patients with systemic sclerosis (SSc).
Methods: SSc patients undergoing screening for pulmonary arterial hypertension (PAH) by echocardiography were identified and their charts were retrospectively reviewed. In all patients, we recorded PASP, pulmonary function, and clinical and laboratory data. PAH was defined as an estimated PASP> or =40 mmHg. In each patient, the PASP values with their corresponding time intervals were fitted to a linear function and the slope of the line was calculated.
Results: Seventy-one patients with at least two echocardiographic studies each were analysed. In 16 (23%) patients, the rate of PASP progression was > or =2.5 mmHg/year whereas in the remaining 55 (77%) patients the rate of progression was <2.5 mmHg/year. In multiple logistic regression analysis, anti-centromere antibodies (ACA) (OR 8.75, CI 1.12-68.38, p = 0.039) and age > or =50 years at diagnosis (OR 8.76, CI 1.28-60.14, p = 0.027) were independently associated with a rise of PASP by > or =2.5 mmHg/year. Baseline forced vital capacity (FVC) <70% (predicted), Raynaud's duration preceding skin manifestations by > or =5 years, and fibrosis on lung computed tomography (CT) were not associated with a rapid rise of PASP (p>0.05).
Conclusions: Old age at diagnosis and ACA are associated with a relatively rapid rise of PASP estimated by echocardiography in SSc. Screening for PAH in these patients may, if followed by right heart catheterization, detect PAH at an earlier stage and guide therapeutic decisions.