SAT0239 The Cumulative Number of Micro-Haemorrhages and Micro-Thrombosis in Nailfold Videocapillaroscopy Is A Good Predictor of Disease Activity in Systemic Sclerosis: A Validation Study of Nemo Score

Abstract Background In previous studies, we demonstrated that the NEMO score, i.e. the cumulative number of microhaemorrhages (MHEs) and microthromboses (MTs), observed in nailfold videocapillaroscopy was a good indicator of the steady state level of disease activity (DA) in patients with systemic sclerosis (SSc) when the European Scleroderma Study Group (EScSG) index was considered the gold standard. Aim of the study To verify whether the NEMO score could be (i) a valid tool to assess DA, even when the modified European Scleroderma Trials and Research (EUSTAR) index was considered to be the comparator, and (ii) a sensitive method to capture the DA overtime changes. Patients and methods The NEMO score and the EScSG and EUSTAR indices were contemporarily assessed at baseline (T0) and after a follow-up of 4–56 months (T1) in 98 patients with SSc. The differences (Δ) between the T1 and T0 values of the NEMO score and the EScSG and EUSTAR indices were calculated and compared to each other. Results NEMO score values were very closely correlated with the corresponding values of the EScSG and EUSTAR indices both at T0 and T1 observations (p < 0.0001 in all cases with the exception of the correlation with EScSG values at T1 (p < 0.03)). The values of the two composite DA indices were also strictly related to each other in both T0 and T1 observations (p < 0.0001). Receiver operating characteristic (ROC) curve analysis showed the NEMO score had a good sensitivity and specificity in classifying patients with a predefined level of DA (scores ≥ 3.0 and ≥ 2.5 for the EScSG and EUSTAR indices, respectively, p < 0.0001 in both cases). Δ values of the NEMO score were significantly correlated with the corresponding values of both the EScSG and EUSTAR indices. Weighted Cohen’s k level of agreement between Δ values of the NEMO score and those of the EScSG and EUSTAR indices was moderate (0.55 and 0.59, respectively). Conclusions NEMO score proves to be a feasible, non-invasive, and valid tool to assess steady state levels and changes over time of DA in patients with SSc. Thus, it can represent an alternative or complementary method to measure this disease status entity in this disorder.

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

10.21203/rs.3.rs-37432/v2 ◽

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.

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

10.21203/rs.3.rs-37432/v1 ◽

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.

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

Arthritis Research & Therapy ◽

10.1186/s13075-020-02342-5 ◽

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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FRI0489 The Number of Micro-Hemorrhages and Micro-Thrombosis Detected by Nailfold Videocapillaroscopy is Predictive of Disease Activity in Patients with Systemic Sclerosis

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2014-eular.3167 ◽

2014 ◽

Vol 73 (Suppl 2) ◽

pp. 564.2-564

Author(s):

D. Sambataro ◽

G. Sambataro ◽

C. Vitali ◽

W. Maglione ◽

E. Zaccara ◽

...

Keyword(s):

Systemic Sclerosis ◽

Disease Activity ◽

Nailfold Videocapillaroscopy

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

10.21203/rs.3.rs-37432/v4 ◽

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.

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

10.21203/rs.3.rs-37432/v3 ◽

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 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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