Significant nailfold capillary loss and late capillaroscopic pattern are associated with pulmonary arterial hypertension in systemic sclerosis

Abstract

Objectives To evaluate differences in nailfold videocapillaroscopy (NVC) findings between systemic sclerosis-SSc patients with and without a diagnosis of pulmonary arterial hypertension (PAH). Methods 110 SSc patients were enrolled in this cross-sectional, case-control, multi-centre study. Patients were divided into cases (SSc-PAH confirmed by right hearth catheterization-RHC) and controls (SSc-nonPAH with low probability of PAH). NVC patterns (early, active, and late) and morphological parameters (microvascular density, non-specific abnormalities, giant capillaries, micro-haemorrhages, avascular areas) were considered using a semiquantitative scoring system. Results SSc-PAH patients showed higher frequencies of late pattern (p < 0.01), non-specific abnormalities (p < 0.01), lower capillary density (p < 0.01), higher avascular areas (p < 0.01), and a higher mean NVC score (p < 0.01). Contrarily, the early/active pattern (p < 0.01) and a higher rate of micro-haemorrhages (p = 0.04) were more frequent in non-PAH patients. By the multivariate analysis, SSc-PAH patients, compared to non-PAH, had more non-specific abnormalities (27/55, 49.1% vs 10/55, 18.2%, adjusted OR: 16.89, 95%CI: 3.06-93.16), a lower capillary density (grade 3, 20/55, 36.4% vs 5/55, 9.1%, adjusted OR: 38.33, 95%CI: 2.34-367.80), and avascular areas (18/55, 32.7% vs 10/55, 18.2%, adjusted OR: 16.90, 95%CI: 2.64-44.35). A correlation was found between the mean pulmonary arterial pressure-mPAP and avascular areas (p < 0.01), capillary density (p < 0.01), and non-specific abnormalities (p < 0.01). A clinical model including the NVC variables may be able to predict the diagnosis of PAH. Conclusions Our results indicate that the distinctive peripheral microcirculatory injury of SSc, i.e capillary loss and morphological abnormalities, appear more severe and pronounced in patients with SSc-PAH.

Low Carbon Monoxide Diffusing Capacity, Patient-Reported Measures and Reduced Nailfold Capillary Density Are Associated with Interstitial Lung Disease in Systemic Sclerosis

“…We found that a reduced DLco of 72.3% exhibits the best probability to be associated with SSc-ILD. Of note, DLco could be reduced if concomitant PAH and/or emphysema are present, but it should be mentioned that in our study, patients with a high probability of PAH according to the DETECT algorithm and/or PAH diagnosed by RHC and/or with chronic lung disease other than SSc-ILD were excluded [11,16,28,31,34]. Questionnaires assessing dyspnoea in SSc-ILD as well as quality of life are used in clinical practice [10,20,35], especially for correlation with the extent of pulmonary involvement [10,20].…”

“…Our results suggest that all patients diagnosed with SSc should receive a comprehensive clinical assessment, including PFTs, assessment of respiratory symptoms and oesophageal involvement, and NVC, to ensure early identification of ILD, especially in those without clear evidence of interstitial disease at HRCT, or without the classic risk factors, or even in situations where a CT scan cannot be performed quickly, providing baseline measurements to compare with upcoming assessments [3,6,7,9]. Alongside more widespread measures in clinical practice, namely DLco and/or patient-reported questionnaires, there are increasing reports indicating that NVC, a safe and validated tool, particularly the highly reliable measurement of capillary density [48], may be incorporated in high-performance algorithms for the early detection of lung involvement in SSc [9,13,28].…”

“…Microhaemorrhages and micro-thrombosis were excluded from the counting process. A consensus concerning image acquisition and analysis, scoring system, reliability of image acquisition and interpretation had already been reached by the authors (RDA, EC, FF) [26,28] (Figure 1).…”

Low-Carbon Monoxide Diffusing Capacity, Patient-Reported Measures and Reduced Nailfold Capillary Density Are Associated with Interstitial Lung Disease in Systemic Sclerosis

“…We found that a reduced DLco of 72.3% exhibits the best probability of being associated with SSc-ILD. Of note, DLco could be reduced if concomitant PAH and/or emphysema are present, but it should be mentioned that in our study, patients with a high probability of PAH according to the DETECT algorithm and/or PAH diagnosed by RHC and/or with chronic lung disease other than SSc-ILD were excluded [ 11 , 16 , 28 , 31 , 34 ].…”

“…Micro-haemorrhages and micro-thrombosis were excluded from the counting process. A consensus concerning image acquisition and analysis, scoring system and reliability of image acquisition and interpretation had already been reached by the authors (RDA, EC, FF) [ 26 , 28 ] ( Figure 1 ).…”