Endothelial Dysfunction and Nailfold Videocapillaroscopy Pattern as Predictors of Digital Ulcers in Systemic Sclerosis: a Cohort Study and Review of the Literature

2015 ◽  
Vol 49 (2) ◽  
pp. 240-252 ◽  
Author(s):  
Ivone Silva ◽  
Andreia Teixeira ◽  
José Oliveira ◽  
Isabel Almeida ◽  
Rui Almeida ◽  
...  
Keyword(s):  
Cohort Study ◽  
Systemic Sclerosis ◽  
Endothelial Dysfunction ◽  
Digital Ulcers ◽  
Review Of The Literature ◽  
Nailfold Videocapillaroscopy

scholarly journals Capillaroscopy as an Outcome Measure for Clinical Trials on the Peripheral Vasculopathy in SSc—Is It Useful?

2010 ◽  
Vol 2010 ◽  
pp. 1-6 ◽  
Author(s):  
Maurizio Cutolo ◽  
Alberto Sulli ◽  
Carmen Pizzorni ◽  
Vanessa Smith
Keyword(s):  
Clinical Trials ◽  
Systemic Sclerosis ◽  
Outcome Measure ◽  
Digital Ulcers ◽  
Nailfold Videocapillaroscopy ◽  
Therapeutic Trials ◽  
Specific Serum ◽  
Pulmonary Arterial ◽  
Microvascular Impairment ◽  
Clinical Conditions

Peripheral microvascular impairment in systemic sclerosis (SSc) may be easily detected and scored in a safe noninvasive way by nailfold videocapillaroscopy (NVC). The paper highlights clinical conditions related to SSc in which NVC may represent an outcome measure of therapeutical interventions, by elaborating on their already assessed relationship with the NVC patterns and eventually scores. The 3 important biological/clinical conditions are: the positivity for SSc-specific serum autoantibodies, the presence of SSc skin digital ulcers (DUs) and of pulmonary arterial hypertension (PAH) SSc associated. In conclusion, to the question if capillaroscopy (NVC) may represent in SSc an outcome measure for clinical trials on the peripheral vasculopathy, based on the growing evidence and our detailed studies, the answer is positive. Recent therapeutic trials in SSc are confirming this role, and the experience is growing rapidly.

scholarly journals High NEMO score values in nailfold videocapillaroscopy are associated with the subsequent development of ischemic digital ulcers in patients with systemic sclerosis.

2020 ◽  
Author(s):  
Nicoletta Del Papa ◽  
Francesca Pignataro ◽  
Wanda Maglione ◽  
Antonina Minniti ◽  
Domenico Sambataro ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Area Under The Curve ◽  
Subsequent Development ◽  
State Level ◽  
Receiver Operating Curve ◽  
Digital Ulcers ◽  
Cumulative Number ◽  
Nailfold Videocapillaroscopy ◽  
Microvascular Changes

Abstract Background Nailfold videocapillaroscopy (NVC) is a feasible method that allows the observation of the microvascular changes that mark the course of systemic sclerosis (SSc). In previous studies we demonstrated that the NEMO score, i.e., the cumulative number of microhaemorrhages and microthromboses, is a good indicator of the steady state level and overtime changes of disease activity (DA) in SSc.Objectives To verify whether high NEMO scores, which mirror a very active microvascular derangement in the fingers, may be associated with the subsequent development of ischemic digital ulcers (IDUs).Methods The NEMO score was assessed at baseline (T0) in 98 patients with SSc, all classified according to the ACR-EULAR criteria. Of them, 90 were females, 48 had the limited and 50 the diffuse cutaneous variant of SSc. Afterwards, the patients were closely followed up for two years, and the appearance of new IDUs recorded at any time of the follow up.The T0-NEMO score values of patients who developed IDUs were compared to those of patients who did not. A receiver operating curve (ROC) was constructed, and the area under the curve (AUC) calculated by plotting the sensitivity and 1-specificity of the different NEMO score values in predicting the subsequent development of IDUs.Results During the follow-up 38 out of 98 patients developed one or more IDUs. The NEMO score at T0 was significantly higher in those who developed IDUs with respect to those who did not [median 14.5 (95%CI 11.0-21.5), and 4.5 (95%CI 4.0-6.0), respectively, p<.0001]. The ROC curve derived from different T0-NEMO score values had an AUC of 0.79 (95%CI 0.69-0.86, p<0.0001)]. A NEMO score of ≥12 had a sensitivity of 83.3% (95%CI 71.5-91.7), and a specificity of 63.2% (95%CI 46.0-78.2), with positive (P) and negative (N) predictive (PV) values of 58.9% (95%CI 44.7-72.2), and 85.6% (71.8-94.4), respectively. A NEMO score of ≥16 had a sensitivity of 95.0% (95%CI 86.1-99.0), and a NPV of 93.4% (77.5-99.2).Conclusions Being a valid tool to measure DA levels in SSc, the NEMO score also appears to be closely related to the subsequent development of IDUs in this disease.

Bosentan in Clinical Practice for Treating Digital and Other Ischemic Ulcers in Spanish Patients with Systemic Sclerosis: IBER-DU Cohort Study

2011 ◽  
Vol 38 (8) ◽  
pp. 1631-1635 ◽  
Author(s):  
JOSÉ ANDRÉS ROMAN IVORRA ◽  
CARMEN PILAR SIMEON ◽  
JUAN JOSÉ ALEGRE SANCHO ◽  
MARIA VICTORIA EGURBIDE ◽  
MARIA JESÚS CASTILLO ◽  
...  
Keyword(s):  
Cohort Study ◽  
Clinical Practice ◽  
Systemic Sclerosis ◽  
Adverse Events ◽  
Clinical Status ◽  
Median Number ◽  
Study Data ◽  
Digital Ulcers ◽  
Main Adverse Event ◽  
Pulmonary Arterial

Objective.To describe treatment outcomes and safety experience with bosentan in patients with systemic sclerosis (SSc) and digital ulcers (DU), in a clinical setting in Spain.Methods.This was a multicenter, noninterventional retrospective cohort study. Data were collected retrospectively from patients with DU, with or without pulmonary arterial hypertension (PAH), who were initiating bosentan therapy in 2003 (n = 26) or 2004 (n = 41) and followed until May 2005. Data were obtained from centers prescribing bosentan. Relevant measures included number of DU, occurrence of new DU, overall DU clinical status (improved, stabilized, worsened), and bosentan-associated adverse events.Results.Sixty-seven patients with SSc and DU or other ulcers were included. PAH was also present in 12 patients (18%). At the start of bosentan treatment, the median number of DU per patient was 3.0. The median change in number of DU was −3.6 and −5.0 at 12 and 24 months, respectively. Sixty-eight percent of the patients did not develop any new DU at 12 months. DU clinical status was reported at 12 months for 22 patients: 18 patients (81.8%) improved and 4 (18.2%) stabilized. The median treatment duration was 13.0 months. The main adverse event was increase of aminotransferase, observed in 5 patients (7%), leading to discontinuation of treatment in 3 patients (4.4%).Conclusion.Previously reported results of bosentan efficacy in DU management are reproducible in clinical practice. This efficacy is maintained in the longterm followup. Bosentan treatment was well tolerated and adverse events were comparable with those observed in previous reports.

scholarly journals AB1115 REAL-WORLD NAILFOLD VIDEOCAPILLAROSCOPY IN A REFERRAL CENTER IN NORTHWESTERN COLOMBIA: A RETROSPECTIVE COHORT STUDY

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1847.1-1847
Author(s):  
D. C. Varela Tabares ◽  
J. Gutiérrez Bolaños ◽  
L. M. Rodríguez Padilla ◽  
M. A. Mesa Navas ◽  
C. J. Velásquez-Franco
Keyword(s):  
Systemic Sclerosis ◽  
Autoimmune Diseases ◽  
Retrospective Cohort ◽  
Antinuclear Antibodies ◽  
Clinical Findings ◽  
Specific Pattern ◽  
Digital Ulcers ◽  
Nailfold Videocapillaroscopy ◽  
Systemic Autoimmune Diseases

Background:Nailfold videocapillaroscopy is a non-invasive tool for the assessment of peripheral microcirculation, and it is useful for the diagnosis and prognosis of systemic autoimmune diseases. Despite its frequent use in clinical practice, the indications of this procedure are not standardized and there is no clear information in real-life about the reasons for remission, the presence of clinical findings of autoimmune diseases during the procedure, the frequency of patterns of autoantibodies and specific capillaroscopic findings.Objectives:To describe the sociodemographic, clinical, paraclinical, and capillaroscopic findings of a cohort of subjects referred to a capillaroscopy service in northwestern Colombia.Methods:We conducted a retrospective cohort study, including subjects from 2015 to 2018. Patients were evaluated by two expert rheumatologists. Variables: Reasons for referral, capillaroscopic patterns at baseline and at 6-month follow-up, presence of clinical findings of systemic autoimmune diseases during the procedure (Raynaud´s phenomenon, puffy fingers, sclerodactyly, pitting scars, digital ulcers, sclerosis cutis, platysma sign, Gottron, and microstomy), along with the pattern and dilution of antinuclear antibodies. Categorical variables were expressed in frequency and percentage and quantitative variables in mean and standard deviation or median with interquartile range, depending on the distribution of the data. Statistical package: SPSS 25. This survey was approved by the institutional Ethics Committee.Results:A total of 392 capillaroscopies were performed, 318 for the first time. The referral reasons for capillaroscopy were: Raynaud`s phenomenon (n=134; 42.1%), connective tissue disease different than systemic sclerosis (SSc) (n=105; 33.1%), and systemic sclerosis (n=79; 24.8%). The baseline capillaroscopic patterns found were: Normal (n=123; 38.7%), non-specific (n=81; 25.5%), SSc (n=90; 28.3%), scleroderma-like (n=24; 7.5%). Among SSc pattern, early (21/90; 23.3%), active, (38/90; 42.2%), and late patterns (31/90; 34.4%) were found. Of the 12 capillaroscopies that presented a non-specific pattern at 6-month follow-up, only one (8.3%) progressed to a systemic sclerosis pattern. In the SSc patterns, the frequency of clinical findings were: sclerodactyly (n=34; 37.8%), Raynaud`s phenomenon (n=26; 28.9%), puffy fingers (n=10; 11.1%), platysma sign (n=10; 11.1%), pitting scars (n=8; 8.9%), digital ulcers (n=8; 8.9%), telangiectasia (n=7; 7.8%), microstomy (n=4; 4.4%), and Gottron (n=1; 1.1%). In the SSc patterns, 42/44 subjects (95,4%) had positive antinuclear antibodies in an mean dilution of 1:320; the most frequent patterns were: centromere (n=27; 64.3%) and nucleolar (n=6; 14-3%).Conclusion:In a real-world setting, the main referral reason to a capillaroscopic center was Raynaud`s phenomenon; more than a third of the subjects had normal capillaroscopic findings; in the subgroup of baseline non-specific pattern, most of them were normal during follow-up; sclerodactyly and Raynaud`s phenomenon were the most frequent clinical findings in patients with systemic sclerosis capillaroscopic patterns.References:[1]Melchor S, et al. Semin Arthritis Rheum. 2016; 46(3): 350-5.[2]Fichel F, et al. Dermatology. 2014;228(4): 360-7Acknowledgments:School of Health Sciences. Universidad Pontificia Bolivariana. Clinica Universitaria Bolivariana. Medellin, ColombiaDisclosure of Interests:None declared

scholarly journals FRI0231 TREATMENT OF DIGITAL ULCERS IN PATIENTS WITH SYSTEMIC SCLEROSIS: PROSPECTIVE COHORT STUDY

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 699.1-699
Author(s):  
S. H. Chang ◽  
J. B. Jun ◽  
Y. J. Lee ◽  
T. Y. Kang ◽  
Y. Park ◽  
...  
Keyword(s):  
Cohort Study ◽  
Systemic Sclerosis ◽  
Medical Treatment ◽  
Digital Ulcers ◽  
Channel Blockers ◽  
Treatment Groups ◽  
Observational Cohort ◽  
The Mean ◽  
Clinically Significant

Background:Digital ulcer (DU) is a common clinical manifestation in patients with systemic sclerosis (SSc). About 70% of patients with SSc experience DUs during the first 10 years, which limit daily activities and may result in digital gangrene or amputation. Several vasoactive/vasodilating agents have been suggested for treatment, but few studies have compared the efficacy of those drugs.Objectives:The objective of our study was to compare the efficacy of medical treatment for SSc related-DUs, focusing on on endothelin receptor antagonist (ERA) and phosphodiesterase-5 inhibitors (PDE5inh).Methods:In this prospective observational cohort study, we recruited patients who had one or more active SSc-related DUs and newly started or changed a medical treatment for SSc-related DUs from 13 medical centers in South Korea. The primary outcome was to compare the time to resolution of cardinal DU (CU) according to the treatments. The secondary outcomes included changes in the size or number of CU and changes in the number of DUs. CU was defined as the most clinically significant DU chosen by the investigators. Patients were followed up at every 4 weeks after enrollment until 12 weeks and finally at 24 weeks.Results:Seventy-one patients were enrolled. Seven patients were excluded due to follow-up loss or withdrawal of consent. A total of 64 patients were analyzed. Seventy-eight percent (n=50) were female. The mean age at enrollment was 49.6 ± 11.6 year-old, and the mean disease duration was 7.1 ± 5.9 years. Twenty-eights pateitns (43.8%) were limited SSc. Forty-nine patients (n=76.6%) started ERA treatment (bosentan=49). Eleven patients (17.2%) started PDE5inh treatment (n=9 for sildenafil, n=1 for udenafil, and n=1 for tadalafil). Four patients who started medication other than ERA or PDE5inh classified as other treatment groups. Seventeen patients (26.6%) were on background calcium channel blockers (CCBs). CU healed in 25 patients (39.1%) at 12 weeks and 43 patients (67.2%) at 24 weeks. The mean time to heal CUs were 54.4 ± 22.7 days at 12 weeks and 91.6 ± 49.2 days at 24 weeks. Time to heal CU was comparable among patients on ERA, PDE5inh, and others (p=0.53, figure 1). The CU area was comparable among the three groups at baseline, 12, and 24 weeks. The mean area of CU in patients on ERA at baseline at 12, and 24 weeks was 21.3±19.4 mm2, 8.2±14.6 mm2, and 4.6±7.7 mm2, respectively. The mean area of CU in patients with PDE5inh at baseline at 12, and 24 weeks was 26.2±28.1 mm2, 3.5±3.6 mm2, and 1.3±4.3 mm2. New DUs developed in 4 patients (8.3%) in ERA, whereas 4 patients (40.0%) in PDE5inh at 4 weeks. The use of ERA was significantly associated with less new DUs development than the use of PDE5inh at 4 weeks follow-up (RR for developing new DU patients on ERA, 0.21; 95% CI 0.06-0.70; p=0.02) At 24 weeks follow-up, none of the patients on CCB developed new DUs.Conclusion:The time to heal CU for ERA and PDEinh users was comparable in the current study. ERA treatment was associated with reduced new DU occurrence compared with PDE5inh treatment. None of the patients with CCB treatment developed new DU development at 24 weeks.Acknowledgments:This study was supported by Handok Pharmaceutical Inc., Seoul, Republic of Korea.Disclosure of Interests:Sung Hae Chang: None declared, Jae-Bum Jun Grant/research support from: Clinical trials; Corbus, JW Pharmaceutical, Speakers bureau: SK Chemical, Yun Jong Lee: None declared, Tae Young Kang: None declared, Yongbeom Park: None declared, Seung-Geun Lee: None declared, Shin-Seok Lee: None declared, Eun Bong Lee: None declaredFigure 1

scholarly journals High NEMO score values in nailfold videocapillaroscopy are predictive of future development of ischemic digital ulcers in patients with systemic sclerosis

2020 ◽  
Author(s):  
Nicoletta Del Papa ◽  
Francesca Pignataro ◽  
Wanda Maglione ◽  
Antonina Minniti ◽  
Domenico Sambataro ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Future Development ◽  
Area Under The Curve ◽  
Subsequent Development ◽  
State Level ◽  
Receiver Operating Curve ◽  
Digital Ulcers ◽  
Cumulative Number ◽  
Nailfold Videocapillaroscopy ◽  
Predictive Instrument

Abstract BackgroundNailfold videocapillaroscopy (NVC) is a feasible method that allows the observation of the microvascular changes that mark the course of systemic sclerosis (SSc). In previous studies we demonstrated that the NEMO score, i.e., the cumulative number of microhaemorrhages and microthromboses, is a good indicator of the steady state level and overtime changes of disease activity (DA) in SSc.ObjectivesTo verify whether high NEMO scores, which mirror a very active microvascular derangement in the fingers, may predict the subsequent development of ischemic digital ulcers (IDUs).Methods The NEMO score was assessed at baseline (T0) in 98 patients with SSc, all classified according to the ACR-EULAR criteria. Of them, 90 were females, 48 had the limited and 50 the diffuse cutaneous variant of SSc. Afterwards, the patients were closely followed up for two years, and the appearance of new IDUs recorded at any time of the follow up.The T0-NEMO score values of patients who developed IDUs were compared to those of patients who did not. A receiver operating curve (ROC) was constructed, and the area under the curve (AUC) calculated by plotting the sensitivity and 1-specificity of the different NEMO score values in predicting the subsequent development of IDUs.Results During the follow-up 38 out of 98 patients developed one or more IDUs. The NEMO score at T0 was significantly higher in those who developed IDUs with respect to those who did not [median 14.5 (95%CI 11.0-21.5), and 4.5 (95%CI 4.0-6.0), respectively, p<.0001]. The ROC curve derived from different T0-NEMO score values had an AUC of 0.79 (95%CI 0.69-0.86, p<0.0001)]. A NEMO score of ≥12 had a sensitivity of 83.3% (95%CI 71.5-91.7), and a specificity of 63.2% (95%CI 46.0-78.2), with positive (P) and negative (N) predictive (PV) values of 58.9% (95%CI 44.7-72.2), and 85.6% (71.8-94.4), respectively. A NEMO score of ≥16 had a sensitivity of 95.0% (95%CI 86.1-99.0), and a NPV of 93.4% (77.5-99.2).ConclusionsBeing a valid tool to measure DA levels in SSc, the NEMO score also appears to be good predictive instrument to predict future development of IDUs in this disease.

Longterm Treatment with Endothelin Receptor Antagonist Bosentan and Iloprost Improves Fingertip Blood Perfusion in Systemic Sclerosis

2014 ◽  
Vol 41 (5) ◽  
pp. 881-886 ◽  
Author(s):  
Maurizio Cutolo ◽  
Barbara Ruaro ◽  
Carmen Pizzorni ◽  
Francesca Ravera ◽  
Vanessa Smith ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Blood Perfusion ◽  
Progressive Increase ◽  
Digital Ulcers ◽  
Endothelin Receptor ◽  
Nailfold Videocapillaroscopy ◽  
Raynaud Phenomenon ◽  
Doppler Flowmetry ◽  
Longterm Treatment ◽  
Capillary Dilation

Objective.To evaluate the longterm effects of endothelin-1 (ET-1) antagonism on peripheral blood perfusion (PBP) in patients with systemic sclerosis (SSc).Methods.Twenty-six patients with SSc already receiving cyclic intravenous iloprost (ILO) for severe Raynaud phenomenon were enrolled. Thirteen patients continued the treatment for a further 3 years (ILO group) and 13 patients, because of the appearance of digital ulcers, received in addition bosentan (BOS; 125 mg twice/day) for 3 years (ILO + BOS group). Both PBP at fingertips and nailfold microangiopathy were evaluated yearly by laser Doppler flowmetry and nailfold videocapillaroscopy, respectively.Results.A progressive significant increase of PBP was observed in the ILO + BOS group during the 3 followup years (p = 0.0007, p = 0.0002, p = 0.01, respectively). In contrast, an insignificant progressive decrease of PBP was observed in the ILO group. Difference of perfusion between the PBP evaluations at basal temperature and at 36°C (to test capillary dilation capacity), was found progressively decreased during the 3-year followup only in the ILO group (p = 0.05, p = 0.26, p = 0.09, respectively). A progressive increase of nailfold capillary number was observed only in the ILO + BOS group after 2 and 3 years of followup (p = 0.05).Conclusion.Longterm treatment of SSc patients with ET-1 antagonism, in combination with ILO, seems to increase fingertip blood perfusion, as well as both capillary dilation capacity and number.

scholarly journals High NEMO score values in nailfold videocapillaroscopy are associated with the subsequent development of ischaemic digital ulcers in patients with systemic sclerosis

2020 ◽  
Vol 22 (1) ◽  
Author(s):  
Nicoletta Del Papa ◽  
Francesca Pignataro ◽  
Wanda Maglione ◽  
Antonina Minniti ◽  
Domenico Sambataro ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Area Under The Curve ◽  
Subsequent Development ◽  
State Level ◽  
Receiver Operating Curve ◽  
Digital Ulcers ◽  
Cumulative Number ◽  
Nailfold Videocapillaroscopy ◽  
Microvascular Changes

Abstract Background Nailfold videocapillaroscopy (NVC) is a feasible method that allows the observation of the microvascular changes that mark the course of systemic sclerosis (SSc). In previous studies, we demonstrated that the NEMO score, i.e. the cumulative number of microhaemorrhages and microthromboses, is a good indicator of the steady-state level and overtime changes of disease activity (DA) in SSc. Objectives To verify whether high NEMO scores, which mirror a very active microvascular derangement in the fingers, may be associated with the subsequent development of ischaemic digital ulcers (IDUs). Methods The NEMO score was assessed at baseline (T0) in 98 patients with SSc, all classified according to the ACR-EULAR criteria. Of them, 90 were females, 48 had the limited and 50 had the diffuse cutaneous variant of SSc. Afterwards, the patients were closely followed up for 2 years, and the appearance of new IDUs recorded at any time of the follow-up. The T0-NEMO score values of patients who developed IDUs were compared to those of patients who did not. A receiver operating curve (ROC) was constructed, and the area under the curve (AUC) calculated by plotting the sensitivity and 1-specificity of the different NEMO score values in predicting the subsequent development of IDUs. Results During the follow-up, 38 out of 98 patients developed one or more IDUs. The NEMO score at T0 was significantly higher in those who developed IDUs with respect to those who did not [median 14.5 (95% CI 11.0–21.5) and 4.5 (95% CI 4.0–6.0), respectively, p < 0.0001]. The ROC curve derived from different T0-NEMO score values had an AUC of 0.79 (95% CI 0.69–0.86, p < 0.0001). A NEMO score of ≥ 12 had a sensitivity of 83.3% (95% CI 71.5–91.7) and a specificity of 63.2% (95% CI 46.0–78.2), with positive (P) and negative (N) predictive (PV) values of 58.9% (95% CI 44.7–72.2) and 85.6% (71.8–94.4), respectively. A NEMO score of ≥ 16 had a sensitivity of 95.0% (95% CI 86.1–99.0) and a NPV of 93.4% (77.5–99.2). Conclusions Being a valid tool to measure DA levels in SSc, the NEMO score also appears to be closely related to the subsequent development of IDUs in this disease.

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