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.

Highlights

  • Nailfold capillaroscopy (NC) is an important tool for the diagnosis of systemic sclerosis (SSc)

  • The median observation time was 1.0 year (IQR 1.0–1.1). Of these 61 patients, 24 patients according to central assessor 1 (39%) and 26 patients according to central assessor 2 (43%) had no megacapillaries present on any assessed finger either at baseline or at the follow-up visit (Table 2)

  • Due to the absence of megacapillaries, the capillaroscopic skin ulcer risk index (CSURI) could not be calculated for those patients

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Summary

Introduction

Nailfold capillaroscopy (NC) is an important tool for the diagnosis of systemic sclerosis (SSc). 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. Systemic sclerosis (SSc) is a chronic connective tissue disease characterised by endothelial cell dysfunction and fibrosis of the skin and internal organs [1, 2]. NC is an important tool for the classification and diagnosis of SSc in clinical practice [5, 6]. The three NC patterns early, active and late were found to be associated with Raynaud’s phenomenon (RP) as well as with the duration of the disease, possibly reflecting SSc evolution [4].

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