Cardiovascular outcome in systemic sclerosis

Objectives Cardiovascular involvement is recognized as a poor prognostic factor in systemic sclerosis (SSc). The aim of this study was to evaluate the usefulness of nailfold video-capillaroscopy (NVC), brain natriuretic peptide (BNP) blood level and exercise echocardiography to predict the occurrence of cardiovascular events in SSc. Methods We prospectively enrolled 65 patients with SSc (age 54 +/- 14 years, 30% female) followed in CHU Sart-Tilman, Liege, Belgium. All patients underwent graded semi-supine exercise echocardiography. Both baseline resting pulmonary hypertension (PH) and PH during follow-up (FUPH) were defined as systolic pulmonary arterial pressure (sPAP) >35 mmHg, and exercise-induced PH (EIPH) as sPAP >50 mmHg during exercise. Results EIPH was present in 21 patients. During FU (27 +/- 18 months), 13 patients developed FUPH and 9 presented cardiovascular complications. Patients with cardiovascular events were significantly older (63 +/- 14 vs 52 +/- 13 years; P = 0.03), presented more frequently NVC grade >2 (89 vs 43%; P = 0.009), had higher resting and exercise sPAP (30 +/- 6 vs 24 +/- 6; P = 0.007 and 57 +/- 13 vs 44 +/- 13 vs mmHg; P = 0.01, respectively), and higher BNP blood level (112 +/- 106 vs 26 +/- 19 pg/ml; P = 0.0001). After adjustment for age and gender, NVC grade >2 (beta = 2.4 +/- 1.1; P = 0.03), EIPH (beta = 2.30 +/- 1.13; P = 0.04), FUPH (beta = 0.24 +/- 0.09; P = 0.01 and beta = 3.52 +/- 1.16; P = 0.002, respectively;) and BNP (beta = 0.08 +/- 0.04; P = 0.02) were independent predictors of CV events. Beyond age, an incremental value of EIPH, BNP and NVC grade >2 was predictive of cardiovascular events (P < 0.001). Conclusion Cardiovascular complications are not rare in SSc (18%). NVC, BNP blood level assessment and exercise echocardiography could be useful tools to identify patients at risk of SSc.