Nailfold Capillaroscopy in Systemic Sclerosis Patients with and without Pulmonary Arterial Hypertension: A Systematic Review and Meta-Analysis

Abstract

Systemic sclerosis (SSc)-related pulmonary arterial hypertension (SSc-PAH) is a leading cause of mortality in SSc. The extent of peripheral microvasculopathy assessed through nailfold capillaroscopy might correlate with the presence of PAH in SSc patients. We searched the PubMed, Cochrane Library, Scopus, and Web of Science databases and performed a random effects meta-analysis of observational studies comparing nailfold capillaroscopic alterations in SSc-PAH versus SSc-noPAH patients. Weighted mean differences (WMD) with the corresponding confidence intervals (CIs) were estimated. The quality of the included studies was evaluated using a modified Newcastle–Ottawa scale. Seven studies with 101 SSc-PAH and 277 SSc-noPAH participants were included. Capillary density was marginally reduced in the SSc-PAH group (WMD: −1.0, 95% CI: −2.0 to 0.0, I2 = 86%). This effect was strengthened once PAH diagnosis was confirmed by right heart catheterization (WMD: −1.2, 95% CI: −2.3 to −0.1, I2 = 85%). An increase in capillary loop width was observed in SSc-PAH compared to SSc-noPAH patients (WMD: 10.9, 95% CI: 2.5 to 19.4, I2 = 78%). Furthermore, SSc-PAH patients had a 7.3 times higher likelihood of active or late scleroderma pattern (95% CI: 3.0 to 18.0, I2 = 4%). SSc-PAH patients presented with worse nailfold capillaroscopic findings compared to SSc-noPAH patients.

Peripheral Microangiopathy Changes in Pulmonary Arterial Hypertension Related to Systemic Sclerosis: Data From a Multicenter Observational Study

“…In addition, low capillary density correlates directly with mean pulmonary pressure. Overall, our findings provide additional evidence to the literature data, confirming that a higher degree of peripheral nailfold microangiopathy is more common in SSc-PAH patients, and further strengthening the concept that NVC changes may run parallel with similar abnormalities inside pulmonary microcirculation ( 23 , 24 ). If confirmed by further investigations in larger patients' series, NVC microvascular alterations could be included in the armamentarium of PAH early detection and so to contribute to a better survival of the patients.…”

“…Previous studies demonstrated some correlations between NVC abnormalities and the severity of internal organs involvement, including PAH (20,23,24,30,31). In a 3year prospective study, the sequential loss of capillaries was recognized as a marker for the occurrence of PAH (31).…”

“…Our data confirmed the higher NVC rating scores more frequent in the SSc using the semi-quantitative assessment, in SSc-PAH group ( Table 2 ) while, using the same scoring method for avascular area, we reported the higher frequency of capillary dropout in SSc-PAH+ patients ( 21 ) as reported in the literature studies ( 21 , 22 , 32 ). We also evaluated the different qualitative patterns across studies ( 21 – 24 , 32 ). Corrado et al ( 22 ) found that the percentage of patients presenting the more severe NVC patterns (active/late) was overall significantly greater in SSc-PAH+ compared to SSc-PAH- (73.2% vs. 50% respectively.…”

“…Nailfold video-capillaroscopy (NVC) is a well-known, validated, noninvasive imaging technique, which allows assessing peripheral microcirculation and diagnosing different diseases affecting peripheral microcirculation (19). Some NVC studies have shown a more severe peripheral microvascular dysfunction in SSc patients with PAH compared to those without PAH, suggesting that abnormalities in peripheral microcirculation may correlate with pulmonary microangiopathy (7,(20)(21)(22)(23)(24).…”

Association Between Uric Acid and Worsening Peripheral Microangiopathy in Systemic Sclerosis

“…"Abnormal nailfold capillaries" (when referring to the "scleroderma pattern") are included in the 2013 American College of Rheumatology (ACR)/ European League Against Rheumatism (EULAR) classification criteria for SSc (11). Cumulative data suggest that NVC measurements can serve as a reliable marker of the extent and severity of microvasculopathy in different vascular districts such as pulmonary (12) and myocardial microcirculation (13) to the point that NVC is currently considered as a surrogate marker of SSc progression (14). However, the association between UA and NVC changes in SSc has not been well-established.…”