The European Scleroderma Trials and Research group (EUSTAR) task force for the development of revised activity criteria for systemic sclerosis: derivation and validation of a preliminarily revised EUSTAR activity index

Valentini, Gabriele; Iudici, Michele; Walker, Ulrich A.; Jaeger, Veronika K.; Baron, Murray; Carreira, Patrícia; Czirják, László; Denton, Christopher P.; Distler, Oliver; Hachulla, É.; Herrick, Ariane L.; Kowal-Bielecka, Otylia; Pope, Janet; Müller‐Ladner, Ulf; Riemekasten, Gabriela; Avouac, Jérôme; Frerix, Marc; Jordan, Suzana; Minier, Tünde; Siegert, Elise; Ong, Voon H; Vettori, Serena; Allanore, Yannick

doi:10.1136/annrheumdis-2016-209768

Cited by 165 publications

(155 citation statements)

References 33 publications

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“…Microvascular dysfunction is a key pathophysiology of SSc . Since at an early stage without internal organ manifestation the disease process is potentially reversible under drug treatment , assessment of the microvascular dysfunction is crucial for individual patient management. However, monitoring of disease activity is still a major challenge for several reasons: First, SSc is a heterogeneous disorder with a high inter‐individual variation regarding disease burden, activity and course .…”

Section: Discussionsupporting

confidence: 93%

“…A minimum of two of the following criteria were mandatory for disease activity to be classified as “active/progressive” disease: worsening of (1) skin involvement, (2) DUs, (3) CRP elevation (>1 mg/dL), and (4) pulmonary symptoms (progressive fibrosis or reduced diffusion capacity) within the past month. In addition, all patients with active disease identified by the use of these 4 criteria would also be classified as active disease as defined by the revised SSc activity index . The study protocol was reviewed by the local ethics committee (Protocol No.…”

Section: Methodsmentioning

confidence: 99%

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Multispectral optoacoustic tomography of systemic sclerosis

Masthoff

Helfen

Claussen

et al. 2018

Journal of Biophotonics

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The study aimed to evaluate the clinical feasibility of hybrid ultrasound/multispectral optoacoustic tomography (MSOT) for assessing microvascular dysfunction in systemic sclerosis (SSc). A handheld US/MSOT imaging system was applied for imaging patients diagnosed with SSc (n = 7) and healthy volunteers (n = 8). Semiquantitative MSOT values for deoxygenated (HbR), oxygenated (HbO ) and total haemoglobin (HbT) were analysed for subcutaneous finger tissue of both hands (8 fingers per subject, 120 fingers in total) and used to assess disease activity (progressive vs stable). Grouped data were compared by one-way nested analysis of variance, Tukey post-hoc test as well as student's t test were used for statistical analysis.Subcutaneous finger tissue of patients with SSc provided significantly lower MSOT values for HbO (26.16 ± 0.71 vs 38.2 ± 1.54, P = .023) and HbT (55.92 ± 1.62 vs 72.46 ± 1.90, P = .018) compared to healthy volunteers. Patients with progressive SSc had significantly lower MSOT values compared to patients with stable disease and healthy volunteers.This pilot study shows the feasibility of MSOT imaging to resolve microvascular dysfunction in SSc as a marker of disease activity. By providing biological tissue properties not revealed by other imaging modalities, MSOT might help to grade SSc non-invasively and monitor early therapy response.

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Section: Discussionsupporting

confidence: 93%

Section: Methodsmentioning

confidence: 99%

Multispectral optoacoustic tomography of systemic sclerosis

Masthoff

Helfen

Claussen

et al. 2018

Journal of Biophotonics

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“…Patients did not receive any disease-modifying antirheumatic drug treatment at the time of biopsy. Active disease was defined according to the European Scleroderma Trials and Research group (EUSTAR) criteria for disease activity 20. Twenty age-matched and sex-matched healthy volunteers served as controls.…”

Section: Methodsmentioning

confidence: 99%

Poly(ADP-ribose) polymerase-1 regulates fibroblast activation in systemic sclerosis

et al. 2018

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“…EULAR Scleroderma Trials and Research Group investigators recently identified and validated a new activity index that performs better than the previous ESSG activity index used in this study and in the previous one [27]. A study has just been set up in our centres to investigate the relationships between the NEMO score and the revised activity index.…”

Section: Discussionmentioning

confidence: 99%

The cumulative number of micro-haemorrhages and micro-thromboses in nailfold videocapillaroscopy is a good indicator of disease activity in systemic sclerosis: a validation study of the NEMO score

et al. 2017

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BackgroundSome abnormalities in nailfold videocapillaroscopy (NVC), such as the presence of micro-haemorrhages (MHEs), micro-thromboses (MTs), giant capillaries (GCs) and reduction in the number of capillaries (nCs), suggest a disease activity (DA) phase in systemic sclerosis (SSc). In a previous paper, we showed that the number of micro-haemorrhages and micro-thromboses (the so-called NEMO score) was the NVC feature more closely associated with DA. The present study was aimed at validating the NEMO score as a measure of DA in patients with SSc.MethodsTwo cohorts of 122 and 97 patients with SSc who were referred to two different rheumatology units, one in Milan and one in Naples, respectively, constituted the validation cohorts. The NEMO score, the total number of GCs and the mean nCs per digit were the parameters defined in each patient by eight-finger NVC. An expert operator analysed the NVCs in each of the participating units. The European Scleroderma Study Group (ESSG) index was used to define the DA level in each patient at the time of NVC examination.ResultsThe NEMO score was the NVC parameter more strictly correlated with the ESSG score in both the Milan and Naples cohorts (p < 0.0001), and it was the only one among the NVC variables that gave a significant contribution in a logistic model where the ESSG score represented the dependent variable. ROC curve analysis confirmed that the NEMO score had the best performance in measuring DA. The AUC of the NEMO score was significantly greater than the AUCs obtained by plotting the sensitivity and specificity of the number of GCs and the mean nCs (p < 0.0001 in all cases). The NEMO score values that showed the best sensitivity-specificity balance in capturing patients with a relevant DA level were slightly higher in the Naples cohort than in the Milan cohort.ConclusionsThis study confirms that the presence of a certain number of MHEs and MTs in NVC may be considered a strong warning signal of a current phase of DA in patients with SSc.

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The European Scleroderma Trials and Research group (EUSTAR) task force for the development of revised activity criteria for systemic sclerosis: derivation and validation of a preliminarily revised EUSTAR activity index

Abstract: A preliminarily revised SSc activity index has been developed and validated, providing a valuable tool for clinical practice and observational studies.

Cited by 165 publications

References 33 publications

Multispectral optoacoustic tomography of systemic sclerosis

Multispectral optoacoustic tomography of systemic sclerosis

Poly(ADP-ribose) polymerase-1 regulates fibroblast activation in systemic sclerosis

The cumulative number of micro-haemorrhages and micro-thromboses in nailfold videocapillaroscopy is a good indicator of disease activity in systemic sclerosis: a validation study of the NEMO score

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