Predictive role of capillaroscopic skin ulcer risk index in systemic sclerosis: a multicentre validation study

Sebastiani, Marco; Manfredi, Andreina; Vukatana, G.; Moscatelli, Sheila; Riato, L.; Bocci, Mario; Iudici, Michele; Principato, Alfonso; Mazzuca, S.; Medico, Patrizia Del; Angelis, Rossella De; D'Amico, Roberto; Vicini, Roberto; Colaci, Michele; Ferri, Clodoveo

doi:10.1136/annrheumdis-2011-200022

Cited by 111 publications

(92 citation statements)

References 16 publications

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“…Indeed, some studies have shown that the late NVC pattern was predictive of the further development of severe peripheral vasculopathy and pulmonary vascular disease (14,20,21), and Sebastiani et al (22,23) showed that capillary loss, one of the main characteristics of the late NVC pattern, was the most relevant parameter to evaluate the risk of developing new digital ulcers of the capillaroscopic skin ulcer risk index formula.…”

Section: Discussionmentioning

confidence: 99%

Skin Telangiectasia and the Identification of a Subset of Systemic Sclerosis Patients With Severe Vascular Disease

Hurabielle

Avouac

Lepri

et al. 2016

Arthritis Care & Research

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Objective. Cutaneous telangiectasia (CT) are common in systemic sclerosis (SSc) patients, but their ability to stratify patients by risk is poorly known. We aimed to determine whether the number and size of CT were associated with the pattern of microvascular lesions assessed by nailfold videocapillaroscopy (NVC) and markers reflecting the severity of SSc-related vasculopathy.Methods. We performed a cross-sectional study, including consecutive SSc patients over a 6-month period. We also considered 3 predefined subsets of patients according to the number of hand or face CT: absence, £10, or >10 hand or face CT (profuse CT). Pseudotumoral CT were defined as CT with >5 mm diameter. Results. A total of 87 patients were included, of whom 75 (86%) had CT (27 with profuse and 19 with pseudotumoral CT). Profuse and pseudotumoral CT were both associated with capillary loss (P < 0.001 and P 5 0.002, respectively) and severe neoangiogenesis (P 5 0.015 and P 5 0.041, respectively), 2 hallmarks of the late NVC pattern. In multivariate analysis, profuse CT were independently associated with past or current digital ulcers (odds ratio [OR] 2.95 [95% confidence interval (95% CI) 1.09-19.63]), and pseudotumoral CT were independently associated with the late NVC pattern ) and with precapillary pulmonary hypertension . Conclusion. We demonstrate that the number and size of CT are associated with the most severe NVC pattern. In addition, profuse and pseudotumoral CT identify a subset of patients with a more severe vascular phenotype. Further prospective studies should determine whether CT number and size could serve as an early clinical biomarker for the development of severe vascular disease.

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Section: Discussionmentioning

confidence: 99%

Skin Telangiectasia and the Identification of a Subset of Systemic Sclerosis Patients With Severe Vascular Disease

Hurabielle

Avouac

Lepri

et al. 2016

Arthritis Care & Research

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“…Ein weiterer Index CSURI (capillaroscopic skin ulcer risk index) soll mit hoher Sensitivität und Spezifität bei Patienten mit SSc das Risiko abschätzen, digitale Ulzerationen zu entwickeln [12]. Der prädiktive Wert des CSURI wurde in einer größeren prospektiven multizentrischen Folgestudie bei einem größeren Patientenkollektiv bestätigt [13]. Einen vereinfachten Algorithmus zur Risikoabschät-zung entwickelten Ingegnoli et al [14] Nicht zuletzt führten die Studienergebnisse der letzten Jahre dazu, dass bei der Consensus-Konferenz der European League Against Rheumatism (EULAR) Scleroderma Trial and Research group (EUSTAR) ein pathologisches Kapillarmuster, neben "puffy fingers", Sklerodaktylie, Raynaud-Syndrom Tabelle 2 Kapillarmikroskopische Muster nach Cutulo et al [7] und Stadien der deutschen Arbeitsgruppe "Kapillarmikroskopie" [9].…”

Section: Systemische Sklerodermieunclassified

Kapillarmikroskopie

Trautinger

2013

J Deutsche Derma Gesell

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ZusammenfassungDie Mikroskopie der Nagelfalzkapillaren findet zunehmenden Einsatz in Dermatologie, Rheumatologie und Angiologie. Herausragende Bedeutung kommt der Kapillarmikroskopie bei der Differenzierung zwischen primärem und sekundärem Raynaud-Syndrom zu. Am besten ist die Bedeutung der Kapillarmikroskopie bei der systemischen Sklerodermie untersucht. Spezifische Muster haben einen hohen positiv prädiktiven Wert für das Entwickeln der Erkrankung. Bei unauffälliger Kapillararchitektur kann eine systemische Sklerodermie nahezu ausgeschlossen werden. PRINCE (prognostic index for nailfold capillaroscopic examination) wurde entwickelt, um das Sklerodermierisiko bei Patienten zu berechnen. CSURI (capillaroscopic skin ulcer risk index) soll mit hoher Sensitivität und Spezifität bei Patienten mit systemischer Sklerose das Risiko abschätzen, digitale Ulzerationen zu entwickeln.Die Studienergebnisse der letzten Jahre führten dazu, dass bei der ConsensusKonferenz der European League Against Rheumatism Scleroderma Trial and Research Group (EUSTAR) ein pathologisches Kapillarmuster als wichtiges Kriterium in den früh-diagnostischen Algorithmus des VEDOSS-Projekts (very early diagnosis of systemic sclerosis) integriert wurde. Es gibt Hinweise, dass kapillarmikroskopische Muster mit Organbeteiligung korrelieren, und die Kapillarmikroskopie könnte daher bei der Früh-erkennung dieser Komplikationen der systemischen Sklerodermie Anwendung finden. SummaryMicroscopy of the nailfold capillaries has found increasing use in dermatology, rheumatology and angiology particularly as an important tool to distinguish between primary and secondary Raynaud disease. The best evidence is available in systemic sclerosis where specific capillaroscopic patterns have a high positive predictive value for the development of the disease. Conversely, a regular capillary pattern rules out systemic sclerosis with high degree of probability. PRINCE (prognostic index for nailfold capillaroscopic examination) was developed to identify patients at high risk of developing systemic sclerosis. CSURI (capillaroscopic skin ulcer risk index) should predict the risk of developing digital ulcers in patients with systemic sclerosis with high specificity and sensitivity.As a consequence of recent results a pathologic capillary pattern was integrated by the EULAR Scleroderma Trials and Research Group (EUSTAR) in the diagnostic algorithm of the VEDOSS-Project (very early diagnosis of systemic sclerosis). Capillary patterns may correlate with visceral involvement and capillaroscopy thus has the potential as a screening tool to enable early diagnosis of organ involvement in systemic sclerosis. Abbildung 2 Frühdiagnostischer Algorithmus der VEDOSS (very early diagnosis of systemic sclerosis). Peter Jung

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“…The best prediction of digital ulceration is provided by the CSURI [15,75]. The predominant item of this score is the capillary density, which can be assessed easily and reproducibly.…”

Section: Usefulness Of Capillaroscopic Scoresmentioning

confidence: 99%

“…For example, in defi ning capillary density, Sebastiani et al counted every crus of the capillary in 1 mm, even in the case of elongations with a loop-whole appearance, which results in an overestimation of capillary density. They also measured the maximal total capillary diameter in the case of ectasia, resulting in an overestimation of giant capillaries [15,16].…”

Section: Normal and Single Pathologic Fi Ndingsmentioning

confidence: 99%

Nailfold capillaroscopy microscopy - an interdisciplinary appraisal

Klein-Weigel

Sunderkötter

Sander

2016

Vasa

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Summary: Nailfold capillaroscopy is a method of great diagnostic value in the differential diagnosis of primary versus secondary Raynaud´s phenomenon, of systemic sclerosis versus other so called connective tissue diseases and of additional diagnostic value in other entities. Rheumatologists, dermatologists, and angiologists in Germany have convened in an interdisciplinary working group in which they synergistically combined their expertise to develop a common nomenclature and standards for the technical performance of nailfold capillary microscopy. The article gives an overview of historical and technical aspects of capillaroscopy, morphologic fi ndings, and disease-specifi c patterns. It also provides a critical appraisal of its signifi cance in the diagnosis and sequelae of these interdisciplinarily-managed diseases including its performance in children and gives an excursion in the potential perspectives of capillaroscopy in less common indications.

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Predictive role of capillaroscopic skin ulcer risk index in systemic sclerosis: a multicentre validation study

Cited by 111 publications

References 16 publications

Skin Telangiectasia and the Identification of a Subset of Systemic Sclerosis Patients With Severe Vascular Disease

Skin Telangiectasia and the Identification of a Subset of Systemic Sclerosis Patients With Severe Vascular Disease

Kapillarmikroskopie

Nailfold capillaroscopy microscopy - an interdisciplinary appraisal

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