SAT0621 Validation Study of Predictive Value of Capillaroscopic Skin Ulcer Risk Index (CSURI) in Scleroderma Patients Treated with Bosentan

Abstract

BackgroundCSURI is a capillaroscopic index, able to identify scleroderma (SSc) patients at high risk for new or non-healing digital ulcers (DU) in the next three months. CSURI has been validated, only in patients not treated with bosentan, in a large multicenter study in 2011.ObjectivesTo evaluate the predictive value of CSURI in SSc patients assuming bosentan for the prevention of DU.MethodsSeventy-six consecutive SSc patients treated with bosentan were enrolled in a multicenter study (F/M 4.4/1; mean age 56.4±13.6 years; diffuse/limited cutaneous subset 30/44). All patients undergone to NVC and CSURI was calculated according to published studies.At baseline all patients had a history of at least one DU in the last year, and 26 patients (30.3%) showed a current DU. At the time of the study 76.3% of patients were also treated with intravenous prostanoids, while no patients was assuming bosentan for pulmonary arterial hypertension.ResultsAfter 3 months from NVC 18/26 patients showed non healing ulcers and 18/76 patients developed new DU. Receiver operator characteristic curve, performed to analyze the prognostic accuracy of CSURI in regard to DU development, is reported in the figure.The area under the curve (AUC) was 0.69 (95% CI 0.57-0.79, p=0.0019) and the higher sensitivity and specificity were observed for a CSURI value of 2.5 (sensitivity 94.4; specificity 57.5; positive and negative likehood ratio 2.22 and 0.097, respectively).At the validated cut-off value of 2.96 sensitivity was 86.1%, specificity 60.0%, positive and negative likehood ratio 2.15 and 0.23, respectively, showing a lower negative predictive value.ConclusionsIn patients treated with bosentan, CSURI shows a lower positive predictive value if compared with SSc population observed in our previous validation study, while the negative predictive value can be considered acceptable.The cause of this different result is not evaluable by our study. SSc peripheral microangiopathy is sustained by a multifactorial process, only partially known, involving a complex cytokines and cells network. In this picture, bosentan could reduce the incidence of new DU, without significantly interfere with the parameters included in CSURI calculation.Some Authors observed a general improvement of NVC parameters in SSc patients treated with bosentan. These data are not in contrast with our study since CSURI calculation is obtained by considering the “worst” capillaroscopic image. Moreover, a longer period of observation as in the other studies should more significantly influence changes in NVC parameters.In our previous studies, CSURI showed a higher predictive value than history of DU in detecting SSc patients at risk for new lesions. Anyway in this study this role of primary prevention cannot be applicable. Moreover, a special attention could be pointed on patients with recurrent DU, regardless specific treatments.A combined approach based on clinical picture and CSURI could probably help in managing therapy of SSc patients with DU, but only prospective clinical trial ...

Automated assessment of absolute nailfold capillary number on videocapillaroscopic images: Proof of principle and validation in systemic sclerosis

“…Recent evidences, in large SSc cohorts, confirmed that the number of capillaries, as detected by NVC, is the most reliable capillaroscopic parameter for prediction of disease progression and detection of effects of therapies . Additionally, capillary density was used for the construction of several scoring systems such as the CSURI, the MES, and the simple day‐to‐day risk index . Other capillaroscopic parameters and derived quantitative indexes to evaluate disease activity in SSc through NVC imaging have been developed.…”

“…very good reliability of the software was confirmed, according to results of single measures ICC, in the difficult SSc image subsets, but also in the easier images obtained from healthy subjects and PRP subject subsets, used as controls.Recent evidences, in large SSc cohorts, confirmed that the number of capillaries, as detected by NVC, is the most reliable capillaroscopic parameter for prediction of disease progression and detection of effects of therapies 4,5,42,43. Additionally, capillary density was used for the construction of several scoring systems such as the CSURI, the MES, and the simple day-to-day risk index 4,28,44. Other…”

Nailfold Capillaroscopy and Clinical Applications in Systemic Sclerosis

“…This study has been validated in a dual center validation study and has been corroborated by the CAP study in which the performance of the scleroderma pattern had the same performance characteristics as counting the numbers of capillaries in one field of finger three of the dominant hand . The common and logical (as SSc is a disease with progressive obliteration of the microcirculation) denominator is that loss of capillaries (qualitatively described as “late” scleroderma pattern or quantitatively as “lowered” number of capillaries) is key criterion in prospective indici to predict new DU in an SSc population (Figure ) . In this way, for example, a patient with no critical digital ischemia at BL and also no DU at BL and a normal mean number of capillaries in the middle finger of the dominant hand is associated with a low risk to develop DU in the next six months.…”

“…This index had a high positive and negative predictive predictivity in a patient population without vasomodifying therapy use (ERA s) at BL . Nevertheless, the good positive predictive values could not be reproduced in real‐life situations in which vasomodulating therapy use is being allowed . A second Belgian‐Italian pilot study investigated whether just counting the numbers of capillaries in eight fields of one linear mm was enough to be used as predictive formula.…”

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

“…In a validation study, Sebastiani et al [ 9 ] demonstrated high predictive values for the development of DUs within 3 months, especially a high negative predictive value of 97%, but also a high positive predictive value of the CSURI of 81% in patients with a history of DUs. However, in another study by Sebastiani et al [ 17 ] a poorer performance of the CSURI with lower predictive values was also observed in a population of SSc patients treated with bosentan. Differences in DU prediction may therefore be explained by differences in vasoactive medications.…”