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

2019 ◽  
Vol 47 (6) ◽  
pp. 888-895 ◽  
Author(s):  
Vanessa Smith ◽  
Amber Vanhaecke ◽  
Els Vandecasteele ◽  
Miguel Guerra ◽  
Sabrina Paolino ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Pulmonary Arterial Hypertension ◽  
Literature Review ◽  
Arterial Hypertension ◽  
Systematic Literature Review ◽  
Capillary Density ◽  
Nailfold Videocapillaroscopy ◽  
Pulmonary Arterial ◽  
Current Screening

Objective.Pulmonary arterial hypertension (PAH) is one of the leading causes of death in systemic sclerosis (SSc). Current screening algorithms are hampered by low positive predictive values. Outcome measures that could add to performance characteristics would be welcome. We aim to evaluate the role of nailfold videocapillaroscopy (NVC) using standardized definitions, in SSc-related PAH (SSc-PAH).Methods.A systematic review to identify original research papers documenting an association between NVC and right heart catheterization-defined SSc-PAH was performed according to the Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA) guidelines. Subsequently, NVC characteristics were subdivided into quantitative (capillary density, dimension, morphology, and hemorrhages), semiquantitative, and qualitative assessment (NVC pattern), according to the definitions of the European League Against Rheumatism Study Group on Microcirculation in Rheumatic Diseases.Results.The systematic search identified 316 unique search results, of which 5 were included in the final qualitative analysis. The occurrence of incident SSc-PAH unequivocally associated in 2 longitudinal studies with progressive capillary loss (p = 0.04 and p = 0.033) and the progression to a severe (active/late) NVC pattern (p = 0.05/0.01 and HR = 5.12, 95% CI 1.23–21.27). In 3 cross-sectional studies, SSc-PAH was found to be unequivocally inversely associated with capillary density (p = 0.001 and p < 0.05) and associated with the presence of a severe NVC pattern (p = 0.03 and p < 0.05).Conclusion.This is the first systematic literature review investigating the role of NVC in SSc-PAH using standardized description, to our knowledge. Unequivocal associations were found between (incident) SSc-PAH and capillary density and NVC pattern. Integration of NVC into current screening algorithms to boost their performance may be a future step.

Screening for pulmonary arterial hypertension in systemic sclerosis: A systematic literature review.

2020 ◽  
Vol 78 ◽  
pp. 17-25 ◽  
Author(s):  
Cosimo Bruni ◽  
Giacomo De Luca ◽  
Maria-Grazia Lazzaroni ◽  
Elisabetta Zanatta ◽  
Gemma Lepri ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Pulmonary Arterial Hypertension ◽  
Literature Review ◽  
Arterial Hypertension ◽  
Systematic Literature Review ◽  
Pulmonary Arterial

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

2008 ◽  
Vol 68 (2) ◽  
pp. 191-195 ◽  
Author(s):  
H M A Hofstee ◽  
A Vonk Noordegraaf ◽  
A E Voskuyl ◽  
B A C Dijkmans ◽  
P E Postmus ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Capillary Density ◽  
Total Width ◽  
Healthy Controls ◽  
Mean Pulmonary Arterial Pressure ◽  
Microvascular Changes ◽  
Pulmonary Arterial ◽  
Disease Specificity

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 µm vs 44.43 µm, 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 = −0.58, p = 0.039) and IPAH (r = −0.67, 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.

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

2021 ◽  
Vol 10 (7) ◽  
pp. 1528
Author(s):  
Ioanna Minopoulou ◽  
Marieta Theodorakopoulou ◽  
Afroditi Boutou ◽  
Alexandra Arvanitaki ◽  
Georgia Pitsiou ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Meta Analysis ◽  
Capillary Density ◽  
Right Heart Catheterization ◽  
Nailfold Capillaroscopy ◽  
Cochrane Library ◽  
Heart Catheterization ◽  
Pulmonary Arterial

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.

scholarly journals The role of TGF-β or BMPR2 signaling pathway-related miRNA in pulmonary arterial hypertension and systemic sclerosis

2021 ◽  
Vol 23 (1) ◽  
Author(s):  
Bei Xu ◽  
Guanhua Xu ◽  
Ye Yu ◽  
Jin Lin
Keyword(s):  
Systemic Sclerosis ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Transforming Growth Factor Beta ◽  
Transforming Growth Factor ◽  
Regulation Of Gene Expression ◽  
Protein Receptor ◽  
Growth Of Knowledge ◽  
Pulmonary Arterial

AbstractPulmonary arterial hypertension (PAH) is a severe complication of connective tissue disease (CTD), causing death in systemic sclerosis (SSc). The past decade has yielded many scientific insights into microRNA (miRNAs) in PAH and SSc. This growth of knowledge has well-illustrated the complexity of microRNA (miRNA)-based regulation of gene expression in PAH. However, few miRNA-related SSc-PAH were elucidated. This review firstly discusses the role of transforming growth factor-beta (TGF-β) signaling and bone morphogenetic protein receptor type II (BMPR2) in PAH and SSc. Secondly, the miRNAs relating to TGF-β and BMPR2 signaling pathways in PAH and SSc or merely PAH were subsequently summarized. Finally, future studies might develop early diagnostic biomarkers and target-oriented therapeutic strategies for SSc-PAH and PAH treatment.

scholarly journals Evidence for the Involvement of Type I Interferon in Pulmonary Arterial Hypertension

2014 ◽  
Vol 114 (4) ◽  
pp. 677-688 ◽  
Author(s):  
Peter M. George ◽  
Eduardo Oliver ◽  
Peter Dorfmuller ◽  
Olivier D. Dubois ◽  
Daniel M. Reed ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Receptor Expression ◽  
Type I ◽  
Type I Ifn ◽  
Endothelin 1 ◽  
Gene And Protein Expression ◽  
Pulmonary Arterial

Rationale : Evidence is increasing of a link between interferon (IFN) and pulmonary arterial hypertension (PAH). Conditions with chronically elevated endogenous IFNs such as systemic sclerosis are strongly associated with PAH. Furthermore, therapeutic use of type I IFN is associated with PAH. This was recognized at the 2013 World Symposium on Pulmonary Hypertension where the urgent need for research into this was highlighted. Objective : To explore the role of type I IFN in PAH. Methods and Results : Cells were cultured using standard approaches. Cytokines were measured by ELISA. Gene and protein expression were measured using reverse transcriptase polymerase chain reaction, Western blotting, and immunohistochemistry. The role of type I IFN in PAH in vivo was determined using type I IFN receptor knockout (IFNAR1 −/− ) mice. Human lung cells responded to types I and II but not III IFN correlating with relevant receptor expression. Type I, II, and III IFN levels were elevated in serum of patients with systemic sclerosis associated PAH. Serum interferon γ inducible protein 10 (IP10; CXCL10) and endothelin 1 were raised and strongly correlated together. IP10 correlated positively with pulmonary hemodynamics and serum brain natriuretic peptide and negatively with 6-minute walk test and cardiac index. Endothelial cells grown out of the blood of PAH patients were more sensitive to the effects of type I IFN than cells from healthy donors. PAH lung demonstrated increased IFNAR1 protein levels. IFNAR1 −/− mice were protected from the effects of hypoxia on the right heart, vascular remodeling, and raised serum endothelin 1 levels. Conclusions : These data indicate that type I IFN, via an action of IFNAR1, mediates PAH.

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