NEMO score in nailfold videocapillaroscopy is a good tool to assess both steady state levels and overtime changes of disease activity in patients with systemic sclerosis: a comparison with the proposed composite indices for this disease status entity

Pignataro, Francesca; Maglione, Wanda; Minniti, Antonina; Sambataro, Domenico; Sambataro, Gianluca; Campanaro, F.; Valentini, Gabriele; Vitali, Claudio; Papa, Nicoletta Del

doi:10.1186/s13075-019-2032-6

Cited by 12 publications

(12 citation statements)

References 27 publications

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“…Each NVC was classified as "early", "active" or "late" according to the method proposed by Cutolo [16]. In addition, we considered the quantitative number of synchronous microhemorrhages, microthrombosis, and giant capillaries according to the number of microhemorrhages (NEMO) score, a validated index to evaluate DA in SSc according to NVC findings [17][18][19].…”

Section: Methodsmentioning

confidence: 99%

Quantification of Ground Glass Opacities Can Be Useful to Describe Disease Activity in Systemic Sclerosis

Sambataro

Pignataro³

et al. 2020

Diagnostics

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Interstitial lung disease (ILD) is the main cause of death in systemic sclerosis (SSc) patients. Usually, patients have lung involvement characterized by ground glass opacities (GGOs), but honeycombing (HC) is also possible. The Wells score is a semi-quantitative index, which is able to assess ILD by distinguishing its main components. The aim of this work is to evaluate the Wells score in relation to the disease activity (DA) index. We enrolled 40 consecutive SSc-ILD patients (26 diffuse cutaneous form, dcSSc, and 14 limited form, lcSSc). All patients were evaluated by the European Scleroderma Study Group (ESSG) index, high-resolution computed tomography, transthoracic echocardiogram, pulmonary function tests (PTSs), and nailfold videocapillaroscopy for the number of microhemorrhages (NEMO) score. In our study, the total extent of ILD (TE-ILD), fibrosis and GGOs correlated with dyspnea (p = 0.03, 0.01 and 0.01 respectively), but not with the ESSG index. Considering only the dcSSc patients, TE-ILD and GGOs correlated with the ESSG index (r = 0.5 p = 0.009), while fibrosis grade correlated with disease duration and systolic pulmonary artery pressure. In conclusion, our data suggest that GGO correlates with DA, while fibrosis may be a sign of disease damage. The quantification of pulmonary involvement using the Wells score can be a useful tool for assessing the appropriate treatment in SSc patients.

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Section: Methodsmentioning

confidence: 99%

Quantification of Ground Glass Opacities Can Be Useful to Describe Disease Activity in Systemic Sclerosis

Sambataro

Pignataro³

et al. 2020

Diagnostics

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“…NVC in SSc has proved to be useful not only in diagnosis but also in prognosis. The Number of microhEMOrrages (NEMO score) is useful to assess the disease activity in SSc, whereas the mean number of capillaries and AAs can be helpful to stratify the risk of developing complications such as DUs [115][116][117][118]. Recently, a pathologic NVC with scleroderma pattern was also found in patients with IIMs without RP [119], and the presence of bushy capillaries was associated with the diagnosis of IIMs [120].…”

Section: First-line Instrumental Examsmentioning

confidence: 99%

Patients with Interstitial Lung Disease Secondary to Autoimmune Diseases: How to Recognize Them?

Sambataro

Pignataro³

et al. 2020

Diagnostics

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The diagnostic assessment of patients with Interstitial Lung Disease (ILD) can be challenging due to the large number of possible causes. Moreover, the diagnostic approach can be limited by the severity of the disease, which may not allow invasive exams. To overcome this issue, the referral centers for ILD organized Multidisciplinary Teams (MDTs), including physicians and experts in complementary discipline, to discuss the management of doubtful cases of ILD. MDT is currently considered the gold standard for ILD diagnosis, but it is not often simple to organize and, furthermore, rheumatologists are still not always included. In fact, even if rheumatologic conditions represent a common cause of ILD, they are sometimes difficult to recognize, considering the variegated clinical features and their association with all possible radiographic patterns of ILD. The first objective of this review is to describe the clinical, laboratory, and instrumental tests that can drive a diagnosis toward a possible rheumatic disease. The secondary objective is to propose a set of first-line tests to perform in all patients in order to recognize any possible rheumatic conditions underlying ILD.

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“…The derived digital images were stored using dedicated software (VideoCap; Scalar Co. Ltd., Tokyo, Japan). One experienced investigator (FP) was responsible for reviewing and scoring the stored NVC images of all the study patients, according to the NEMO de nition (8).…”

Section: Nailfold Videocapillaroscopymentioning

confidence: 99%

“…During the last few years the NEMO score, i.e., the cumulative number of MHEs and MTs, has been proposed and validated as an good tool to assess both steady state (6,7) and overtime changes (8) of disease activity (DA) in the course of SSc. The NEMO score, in fact, showed a very close correlation with the composite scales which have been de ned to assess this disease status entity, as those proposed by the European Scleroderma Study Group (EScSG) (9,10) and, subsequently, by the European Scleroderma Clinical Trials and Research (EUSTAR) (11).…”

Section: Introductionmentioning

confidence: 99%

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

Papa

Pignataro

Maglione

et al. 2020

Preprint

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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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NEMO score in nailfold videocapillaroscopy is a good tool to assess both steady state levels and overtime changes of disease activity in patients with systemic sclerosis: a comparison with the proposed composite indices for this disease status entity

Cited by 12 publications

References 27 publications

Quantification of Ground Glass Opacities Can Be Useful to Describe Disease Activity in Systemic Sclerosis

Quantification of Ground Glass Opacities Can Be Useful to Describe Disease Activity in Systemic Sclerosis

Patients with Interstitial Lung Disease Secondary to Autoimmune Diseases: How to Recognize Them?

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

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