SHORT TERM NAILFOLD VIDEOCAPILLAROSCOPY EVALUATION MAY NOT REFLECT DIGITAL ULCERS STATUS CHANGES IN PATIENTS WITH SYSTEMIC SCLEROSIS

Author(s):  
Cosimo Bruni
2010 ◽  
Vol 2010 ◽  
pp. 1-6 ◽  
Author(s):  
Maurizio Cutolo ◽  
Alberto Sulli ◽  
Carmen Pizzorni ◽  
Vanessa Smith

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.


2020 ◽  
Author(s):  
Nicoletta Del Papa ◽  
Francesca Pignataro ◽  
Wanda Maglione ◽  
Antonina Minniti ◽  
Domenico Sambataro ◽  
...  

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.


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

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


2020 ◽  
Author(s):  
Nicoletta Del Papa ◽  
Francesca Pignataro ◽  
Wanda Maglione ◽  
Antonina Minniti ◽  
Domenico Sambataro ◽  
...  

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.


2014 ◽  
Vol 41 (5) ◽  
pp. 881-886 ◽  
Author(s):  
Maurizio Cutolo ◽  
Barbara Ruaro ◽  
Carmen Pizzorni ◽  
Francesca Ravera ◽  
Vanessa Smith ◽  
...  

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.


2020 ◽  
Vol 22 (1) ◽  
Author(s):  
Nicoletta Del Papa ◽  
Francesca Pignataro ◽  
Wanda Maglione ◽  
Antonina Minniti ◽  
Domenico Sambataro ◽  
...  

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.


2020 ◽  
Author(s):  
Nicoletta Del Papa ◽  
Francesca Pignataro ◽  
Wanda Maglione ◽  
Antonina Minniti ◽  
Domenico Sambataro ◽  
...  

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.


2020 ◽  
Author(s):  
Nicoletta Del Papa ◽  
Francesca Pignataro ◽  
Wanda Maglione ◽  
Antonina Minniti ◽  
Domenico Sambataro ◽  
...  

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 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).ConclusionsBeing 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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