Abstract
BackgroundTo determine whether lung involvement is related to microvascular perturbations, nailfold videocapillaroscopy (NVC) was performed in patients with systemic sclerosis (SSc).MethodsA cross-sectional study was consecutively accomplished in 152 SSc patients. NVC, a pulmonary function test and echocardiography were undergone within a 3-month period. Finally, 134 patients with at least eight NVC (200× magnification) images were selected for quantitative and qualitative examinations.ResultsPatients with interstitial lung disease presented lower median capillary density (4.86/mm vs 5.88/mm, p = 0.005) and higher median of neoangiogenesis (0.56/mm vs 0.31/mm, p = 0.005). A higher quantity of neoangiogenesis capillaries was found in patients with pulmonary arterial hypertension (0.70/mm vs 0.33/mm, p = 0.008). Multivariate linear regression analysis established a correlation between neoangiogenesis and decreased forced vital capacity (FVC) (p < 0.001): for each capillary with neoangiogenesis visualized on average per 1 mm, FVC was 7.3% reduced. In qualitative NVC, a late pattern as defined by Cutolo was also associated with lower FVC (p = 0.018). The number of giant capillaries was associated with reduced diffusion capacity of the lung for carbon monoxide (DLCO) (p = 0.016); for each giant capillary per 1 mm, DLCO was 11.8% diminished.ConclusionsA good correlation was observed between distinctive quantitative and qualitative NVC features with lung functional parameters such as FVC and DLCO. It is suggested that vasculopathy could play a role in SSc lung involvement.
Highlights
To determine whether lung involvement is related to microvascular perturbations, nailfold videocapillaroscopy (NVC) was performed in patients with systemic sclerosis (SSc)
The lung is one of the most frequently jeopardized organs in SSc; interstitial lung disease (ILD) affects around 55–65% of patients subjected to high-resolution computed tomography (HRCT) [5, 6], and pulmonary arterial hypertension (PAH) diagnosed by right heart catheterization (RHC) involves approximately 10% of this population [7, 8]
The late pattern was related to lower levels of circulating endothelial progenitor cells and higher vascular endothelial growth factor (VEGF), demonstrating that an impairment of vasculogenesis mediates the loss of capillaries [15]
Summary
To determine whether lung involvement is related to microvascular perturbations, nailfold videocapillaroscopy (NVC) was performed in patients with systemic sclerosis (SSc). Systemic sclerosis (SSc) is a connective tissue disease characterized by microvascular damage. It seems that endothelial activation induces the release of inflammatory mediators, platelet activation and inflammatory cell recruitment [1,2,3]. Maricq et al [12] defined the scleroderma pattern as a group of capillaroscopy findings with potential diagnostic value in this disease. Using nailfold videocapillaroscopy (NVC), Cutolo et al [14] defined a new classification of qualitative capillaroscopy abnormalities into early, active and late scleroderma patterns. No prior studies were focused on extensive quantitative NVC analysis and its correlation with objective lung parameters
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