Nailfold capillary density is associated with the presence and severity of pulmonary arterial hypertension in systemic sclerosis

Abstract

Objective: The aim of this study was to investigate whether there are differences in capillary nailfold changes in patients with systemic sclerosis (SSc) with and without pulmonary arterial hypertension (PAH), and whether these changes are associated with PAH severity and disease specificity. Methods: Capillary density and loop dimensions were studied in 21 healthy controls, 20 patients with idiopathic PAH (IPAH) and 40 patients with SSc. Of the 40 patients with SSc, 19 had no PAH (SSc-nonPAH) and 21 had PAH (SSc-PAH), of whom eight had PAH during exercise. Results: Capillary density was lower in SSc-PAH compared with patients who had SSc-nonPAH (4.33/mm vs 6.56/mm respectively, p = 0.001), but loop dimensions were equal. In comparison with IPAH, patients with SSc-PAH had reduced capillary density (4.33/mm vs 7.86/mm, p,0.001) and larger loop dimensions (total width 101.05 mm vs 44.43 mm, p,0.001). Capillary density in healthy controls (9.87/mm) was significantly higher when compared with SSc-nonPAH (6.56/mm), SSc-PAH (4.33/ mm) and with IPAH (7.86/mm). No differences in capillary dimensions were present between healthy controls and IPAH. Capillary density correlated with mean pulmonary arterial pressure (PAP) at rest in SSc-PAH at rest (r = 20.58, p = 0.039) and IPAH (r = 20.67, p = 0.001). Conclusions: Reduction of nailfold capillary density, but not capillary loop dimensions is associated with PAH, and correlates with the severity of PAH in both SSc and IPAH. This suggests that either systemic microvascular changes play a part in the development of PAH, or that PAH itself contributes to systemic microvascular changes.

Survival in patients with pulmonary arterial hypertension associated with systemic sclerosis from a Swedish single centre: prognosis still poor and prediction difficult

“…A reduction of nailfold capillary density has been observed in PAH patients but it is unknown if this actually precedes or accompanies the development of PAH [9]. The presence of anti-centromere antibodies (ACA) [10], ischemic ulcers of the fingers [11], low diffusing capacity of the lung for carbon monoxide (DL CO ) [12,13], high N-teminal pro-brain natriuretic peptide (NTProBNP) [12,14], and increased numbers of teleangiectases [15] have been associated with the development of SSc-PAH whereas cyclic treatment with iloprost infusion has been reported to be preventive in a preliminary study [16].…”

Nailfold capillaroscopy in systemic sclerosis: Data from the EULAR scleroderma trials and research (EUSTAR) database

“…In order to clarify with further aspects the reported associations between nailfold capillary abnormalities and clinical and/or laboratory features (Bredemeier et al, 2004;Caramaschi et al, 2007;Chen et al, 1984;Cutolo et al, 2004;Hofstee et al, 2009;Lovy et al, 1985;Smith et al, 2012;Sulli et al, 2012) it was decided to evaluate a large multinational group of patients with SSc such as the international multicenter cohort of the EULAR Scleroderma Trials And Research (EUSTAR) registry.…”

Training in Capillaroscopy: A Growing Interest: Figure 1.

“…Nailfold capillary density, for example, is associated with the presence and severity of pulmonary arterial hypertension in SSc or with progression of digital ulcers 19,20,21 .…”