Prospective evaluation of the capillaroscopic skin ulcer risk index in systemic sclerosis patients in clinical practice: a longitudinal, multicentre study

Abstract

BackgroundNailfold capillaroscopy (NC) is an important tool for the diagnosis of systemic sclerosis (SSc). The capillaroscopic skin ulcer risk index (CSURI) was suggested to identify patients at risk of developing digital ulcers (DUs). This study aims to assess the reliability of the CSURI across assessors, the CSURI change during follow-up and the value of the CSURI in predicting new DUs.MethodsThis multicentre, longitudinal study included SSc patients with a history of DUs. NC images of all eight fingers were obtained at baseline and follow-up and were separately analysed by two trained assessors.ResultsSixty-one patients were included (median observation time 1.0 year). In about 40% of patients (assessor 1, n = 24, 39%; assessor 2, n = 26, 43%) no megacapillary was detected in any of the baseline or follow-up images; hence the CSURI could not be calculated.In those 34 patients in whom CSURI scores were available from both assessors (26% male; median age 57 years) the median baseline CSURI was 5.3 according to assessor 1 (IQR 2.6–16.3), increasing to 5.9 (IQR 1.3–12.0) at follow-up. According to assessor 2, the CSURI diminished from 6.4 (IQR 2.4–12.5) to 5.0 (IQR 1.7–10.0).The ability of a CSURI ≥ 2.96 category to predict new DUs was low (for both assessors, positive predictive value 38% and negative predictive value 50%) and the inter-assessor agreements for CSURI categories were fair to moderate.ConclusionsIn this study, around 40% of patients could not be evaluated with the CSURI due to the absence of megacapillaries. Clinical decisions based on the CSURI should be made with caution.Trial registrationCurrent Controlled Trials, ISRCTN04371709. Registered on 18 March 2011.Electronic supplementary materialThe online version of this article (10.1186/s13075-018-1733-6) contains supplementary material, which is available to authorized users.

The first composite score predicting Digital Ulcers in systemic sclerosis patients using Clinical data, Imaging and Patient history—CIP-DUS

“…First, CSURI was developed, which identifies patients at risk of developing new DU in a 3-month follow-up [3][4][5]. However, a multicentre study showed that 40% of their patients were not assessable with CSURI due to the absence of megacapillaries, which are an essential part of the index's equation [6]. Furthermore, Caramaschi et al developed a score of risk factors that was associated with ischemic digital ulcers in SSc patients undergoing Iloprost treatment.…”

Kapillarmikroskopie – Grundlagen und klinische Anwendung

“…Für die SSc wurde der „capillaroscopic skin ulcer risk index“ als prognostischer Verlaufsscore entwickelt und validiert, konnte sich aber in multizentrischen Studien nicht bestätigen lassen [ 10 , 11 , 12 ]. Die Kapillardichte (in Kapillaren/mm Nagelfalz) bleibt wichtigster prognostischer Marker [ 13 , 14 , 15 ].…”

Evaluation of cutaneous microcirculation in systemic sclerosis. An update

“…Wartość CSURI obliczana jest na podstawie kilku czyn- ników: liczby kapilar w dystalnym rzędzie w obrębie wału paznokciowego oraz liczby i najszerszego wymiaru megakapilar w ocenie kapilaroskopowej. W niedawnych badaniach autorzy zakwestionowali jednak wartość prognostyczną indeksu CSURI ze względu na ryzyko błędów związanych z samym badaniem oraz stosowanymi metodami analizy statystycznej [14].…”