Prediction of new onset of resting pulmonary arterial hypertension in systemic sclerosis.

Summary Background Early detection of pulmonary arterial hypertension (PH) is crucial in systemic scleroderma. However, predictors of new onset of resting PH during follow-up (FUPH) have been poorly explored. Aim To determine whether nailfold videocapillaroscopy (NVC) grade and exercise echocardiographic variables are predictors of FUPH. Methods We prospectively enrolled 40 patients with systemic sclerosis (age 54 ± 13 years; 68% women). All patients underwent graded semisupine exercise echocardiography and NVC. Baseline resting PH and FUPH were defined as systolic pulmonary arterial pressure (sPAP) > 35 mmHg, and exercise-induced PH (EIPH) as exercise sPAP > 50 mmHg. Results Seventeen patients developed EIPH (43%). During follow-up (FU) (25 ± 15 months), 11 patients without baseline PH developed FUPH (28%), all from the EIPH group. Patients with FUPH were significantly older (60 ± 14 vs 50 ± 12 years; P  = 0.04), had higher resting and exercise sPAP (30 ± 4 vs 22 ± 5 and 60 ± 12 vs 40 ± 11 mmHg, respectively; P P  = 0.0003) and presented more frequently NVC grade > 2 (90% vs 35%; P  = 0.0009). After adjustment for age, resting sPAP, exercise sPAP and NVC grade > 2 were associated with maximal resting sPAP during follow-up and FUPH ( P  2 had a very high incidence of FUPH (82%), and both variables remained strongly associated with FUPH after adjustment for age (hazard ratio 11.6, 95% confidence interval 2.4–55.3; P  = 0.002). Conclusion Exercise echocardiography and NVC can identify a subgroup of patients with systemic sclerosis who are at risk of developing FUPH.