Abstract

Introduction. Systemic sclerosis (SSc) is a rare connective tissue disease. Interstitial lung disease and pulmonary arterial hypertension (PAH) are the main features of SSc. Transthoracic echocardiography is a common technique for screening PAH, but right heart catheterization (RHC) is required to confirm the diagnosis (1). Several studies have shown that nail fold videocapillaroscopy (NVC), the validated tool to study microvascular damage, can predict lung involvement (1). Objective. To evaluate the possible correlation between pulmonary vascular involvement and peripheral microvascular damage in SSc patients. Methods. We enrolled 18 patients with SSc (EULLAR/ACR 2013 criteria) (2). All SSc patients were evaluated by RHC and NVC. Results. We identified 9 patients with PAH (F/M 7/2; median age 68± 8 SD, in years) and 9 without PAH (non-PAH, F/M 7/2; median age 67±9 SD, in years) by RHC evaluation. We observed no statistically significant differences in clinical and laboratory characteristics between PAH and non-PAH. We evaluated a statistically significant correlation between the value of pulmonary vascular resistance at RHC and the number of capillaries at NVC (p=0.04). We observed a statistically significant reduction in capillary density in patients with PAH-SSc compared with those without PAH (p<0.03). In contrast, avascular areas were more frequent in the PAH group (p<0.02) than in the non-PAH group. Conclusions. This study supports the correlation between pulmonary circulation and peripheral microvascular changes. References. 1. Ruaro B et al. Diagnostics (Basel),2022,12:616. 2. van den Hoogen F et al. Ann Rheum Dis, 2013,72:1747-55.