Nailfold Videocapillaroscopy in Systemic Sclerosis–related Pulmonary Arterial Hypertension: A Systematic Literature Review

Smith, Vanessa; Vanhaecke, Amber; Vandecasteele, Els; Guerra, Miguel; Paolino, Sabrina; Melsens, Karin; Cutolo, Maurizio

doi:10.3899/jrheum.190296

Cited by 26 publications

(29 citation statements)

References 37 publications

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“…As a result, capillary density was not the only capillaroscopic parameter linked with SSc-PAH, but we further expanded previous findings by establishing increased capillary loop width and severe scleroderma pattern as markers of SSc-PAH. Given the scarcity of available evidence resulting from the rarity of the disease itself, our meta-analysis, on top of previous studies [18,19], further reinforces the hypothesis that nailfold capillaroscopy might be a useful tool for PAH risk stratification in current rheumatology clinical practice.…”

Section: Strengths and Limitationssupporting

confidence: 74%

“…To our knowledge, this systematic review and meta-analysis provides the most up-todate and comprehensive analysis of the effect of PAH on the peripheral microcirculation in SSc patients. Previous secondary studies have evaluated the use of nailfold capillaroscopy in SSc-PAH [18,19]. However, our study is the first to provide a direct comparison between SSc-PAH and SSc-noPAH individuals, demonstrating that SSc-PAH is characterized by progressing peripheral microvascular changes compared to SSc-noPAH.…”

Section: Strengths and Limitationsmentioning

confidence: 81%

“…Subsequently, the diagnostic accuracy of nailfold videocapillaroscopy [18] as well as its potential role as a screening tool in SSc-PAH have been previously evaluated [19] in systematic reviews, both of which indicated reduced capillary density as a marker of pulmonary vasculopathy. However, no meta-analysis has yet explored the whole spectrum of nailfold capillaroscopic changes in SSc-PAH compared to SSc-noPAH patients.…”

Section: Introductionmentioning

confidence: 99%

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

Minopoulou

Theodorakopoulou

Boutou

et al. 2021

JCM

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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.

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Section: Strengths and Limitationssupporting

confidence: 74%

Section: Strengths and Limitationsmentioning

confidence: 81%

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

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

Minopoulou

Theodorakopoulou

Boutou

et al. 2021

JCM

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“…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.…”

Section: Discussionsupporting

confidence: 87%

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

Giuggioli

Riccieri

Cipolletta

et al. 2022

Front. Cardiovasc. Med.

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Systemic sclerosis (SSc) is a connective tissue disease characterized by immune-system alterations, fibrosis involving the skin and internal organs and diffuse microangiopathy. Pulmonary arterial hypertension (PAH) is a severe complication of SSc affecting about 10–15% of the patients and it is a leading cause of mortality. Due to the devastating nature of SSc-PAH, there is a clear need to systematically adopt appropriate screening programs. Nail fold videocapillaroscopy (NVC) studies have shown a more severe peripheral microvascular dysfunction in SSc patients with PAH suggesting that abnormalities in peripheral microcirculation may correlate with pulmonary microangiopathy. This is a cross-sectional study involving four tertiary University Rheumatology Units in the Center-North of Italy. Seventy patients, 35 adults with SSc and PAH confirmed by RHC (F/M 34/1; median age 65.2 ± 8.9 SD yrs), and 35 SSc patients without PAH were enrolled (F/M 3471; median age 63.3 ± 10.3 SD yrs). Clinical, laboratoristic and instrumental data were collected and NVC was performed in all patient. Specific NVC parameters were evaluated and a semi-quantitative rating scale was adopted to score these changes. Finally, patients were distributed into the suitable NVC pattern belonging to the scleroderma pattern. Our aim was to compare the peripheral microangiopathy changes in SSc patients with and without PAH, and to investigate the relationship between NVC findings and the main hemodynamic parameters of pulmonary vasculopathy. Patients with SSc-PAH+ showed a significant higher frequency of interstitial lung disease (ILD). No significant differences regarding clinical and laboratoristic parameters were observed. NVC abnormalities, avascular areas were more frequent in SSc patients with PAH, respect to those without (p = 0.03), and capillary density was significantly lower when considering grade 3 (p = 0.02). A higher NVC semiquantitative mean was found in SSc-PAH+ patients and a greater rate of the “late” pattern was detected in SSc-PAH+ subjects in respect to PAH- (57.1% vs. 25.7%) (p = 0.03). A significant correlations between pulmonary pressure values (sPAP by TTE and mPAP by RHC) and the capillary density (Spearman's rho 0.35, p = 0.04 for both). 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.

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“…Furthermore, there is strong evidence supporting an association between NVC abnormalities (particularly capillary loss) and disease severity (particularly vascular manifestations such as DU, calcinosis and pulmonary arterial hypertension) 56,57 . In a recent study, the reduction of the number of capillaries on NVC in SSc patients over time was associated with overall disease progression, occurrence of new DU, progression in vascular changes of the lungs (i.e.…”

Section: Nailfold (Video)capillaroscopymentioning

confidence: 99%

The importance of skin manifestations, serology and nailfold (video)capillaroscopy in morphea and systemic sclerosis: current understanding and new insights

Mostmans

Richert

Badot

et al. 2020

Acad Dermatol Venereol

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Since the field around morphea and systemic sclerosis (SSc) is evolving rapidly, this review approaches conventional as well as more recent clinical developments from a dermatological point of view. Skin manifestations are critical in sub‐classifying these diseases ensuring a correct prognosis for these patients. They can be discretely present, and therefore, diagnosis can be challenging sometimes, implicating a thorough dermatological examination is mandatory. Furthermore, a growing amount of dermatologists perform nailfold videocapillaroscopy (NVC), a more recent reliable non‐invasive imaging technique used for in vivo assessment of the microcirculation at the nailfold. After all, specific NVC‐changes are present in a majority of patients with SSc. This way, dermatologists not only take part in the diagnosis process through clinical investigation but also through the use of a modern state of the art imaging technique that is becoming the golden standard in SSc multidisciplinary workup. In this review, current understandings for NVC in morphea and SSc are revised. So far, the role of NVC in the diagnosis/prognosis/classification of morphea patients has not been thoroughly investigated to make proper conclusions. As for SSc, it is well known that NVC contributes to the diagnosis and can make a fundamental difference especially when obvious clinical SSc signs are absent. This review emphasizes the (somewhat underestimated) role of dermatologists in the process of diagnosis and follow‐up, and thus, the difference we can make for our patients and fellow colleagues in the multidisciplinary workup of SSc and morphea.

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Nailfold Videocapillaroscopy in Systemic Sclerosis–related Pulmonary Arterial Hypertension: A Systematic Literature Review

Cited by 26 publications

References 37 publications

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

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

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

The importance of skin manifestations, serology and nailfold (video)capillaroscopy in morphea and systemic sclerosis: current understanding and new insights

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