Skip to main content
Top
Published in: Arthritis Research & Therapy 1/2018

Open Access 01-12-2018 | Research article

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

Authors: Ulrich A. Walker, Veronika K. Jaeger, Katharina M. Bruppacher, Rucsandra Dobrota, Lionel Arlettaz, Martin Banyai, Jörg Beron, Carlo Chizzolini, Ernst Groechenig, Rüdiger B. Mueller, François Spertini, Peter M. Villiger, Oliver Distler

Published in: Arthritis Research & Therapy | Issue 1/2018

Login to get access

Abstract

Background

Nailfold 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.

Methods

This 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.

Results

Sixty-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.

Conclusions

In 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 registration

Current Controlled Trials, ISRCTN04371709. Registered on 18 March 2011.
Appendix
Available only for authorised users
Literature
1.
go back to reference Gabrielli A, Avvedimento EVEV, Krieg T. Scleroderma. N Engl J Med. 2009;360(19):1989–2003.CrossRef Gabrielli A, Avvedimento EVEV, Krieg T. Scleroderma. N Engl J Med. 2009;360(19):1989–2003.CrossRef
2.
go back to reference Varga J, Trojanowska M, Kuwana M. Pathogenesis of systemic sclerosis: recent insights of molecular and cellular mechanisms and therapeutic opportunities. J Scleroderma Relat Disord. 2017;2(3):137–52.CrossRef Varga J, Trojanowska M, Kuwana M. Pathogenesis of systemic sclerosis: recent insights of molecular and cellular mechanisms and therapeutic opportunities. J Scleroderma Relat Disord. 2017;2(3):137–52.CrossRef
3.
go back to reference Cutolo M, Sulli A, Smith V. Assessing microvascular changes in systemic sclerosis diagnosis and management. Nat Rev Rheumatol. 2010;6(10):578–87.CrossRef Cutolo M, Sulli A, Smith V. Assessing microvascular changes in systemic sclerosis diagnosis and management. Nat Rev Rheumatol. 2010;6(10):578–87.CrossRef
4.
go back to reference Cutolo M, Pizzorni C, Secchi ME, Sulli A. Capillaroscopy. Best Pract Res Clin Rheumatol. 2008;22(6):1093–108.CrossRef Cutolo M, Pizzorni C, Secchi ME, Sulli A. Capillaroscopy. Best Pract Res Clin Rheumatol. 2008;22(6):1093–108.CrossRef
5.
go back to reference van den Hoogen F, Khanna D, Fransen J, Johnson SR, Baron M, Tyndall A, et al. 2013 classification criteria for systemic sclerosis: an American College of Rheumatology/European League against Rheumatism collaborative initiative. Arthritis Rheum. 2013;65(11):2737–47.CrossRef van den Hoogen F, Khanna D, Fransen J, Johnson SR, Baron M, Tyndall A, et al. 2013 classification criteria for systemic sclerosis: an American College of Rheumatology/European League against Rheumatism collaborative initiative. Arthritis Rheum. 2013;65(11):2737–47.CrossRef
6.
go back to reference Smith V, Thevissen K, Trombetta AC, Pizzorni C, Ruaro B, Piette Y, et al. Nailfold capillaroscopy and clinical applications in systemic sclerosis. Microcirculation. 2016;23(5):364–72.CrossRef Smith V, Thevissen K, Trombetta AC, Pizzorni C, Ruaro B, Piette Y, et al. Nailfold capillaroscopy and clinical applications in systemic sclerosis. Microcirculation. 2016;23(5):364–72.CrossRef
7.
go back to reference Cutolo M, Smith V. Nailfold capillaroscopy. In: Scleroderma. Boston: Springer US; 2012. p. 331–46.CrossRef Cutolo M, Smith V. Nailfold capillaroscopy. In: Scleroderma. Boston: Springer US; 2012. p. 331–46.CrossRef
8.
go back to reference Sebastiani M, Manfredi A, Colaci M, D’amico R, Malagoli V, Giuggioli D, et al. Capillaroscopic skin ulcer risk index: a new prognostic tool for digital skin ulcer development in systemic sclerosis patients. Arthritis Rheum. 2009;61(5):688–94.CrossRef Sebastiani M, Manfredi A, Colaci M, D’amico R, Malagoli V, Giuggioli D, et al. Capillaroscopic skin ulcer risk index: a new prognostic tool for digital skin ulcer development in systemic sclerosis patients. Arthritis Rheum. 2009;61(5):688–94.CrossRef
9.
go back to reference Sebastiani M, Manfredi A, Vukatana G, Moscatelli S, Riato L, Bocci M, et al. Predictive role of capillaroscopic skin ulcer risk index in systemic sclerosis: a multicentre validation study. Ann Rheum Dis. 2012;71(1):67–70.CrossRef Sebastiani M, Manfredi A, Vukatana G, Moscatelli S, Riato L, Bocci M, et al. Predictive role of capillaroscopic skin ulcer risk index in systemic sclerosis: a multicentre validation study. Ann Rheum Dis. 2012;71(1):67–70.CrossRef
10.
go back to reference Masi AT, Rodnan GP, Medsger TA Jr, Altman RD, D’Angelo WA, Fries JF, et al. Preliminary criteria for the classification of systemic sclerosis (scleroderma). Subcommittee for scleroderma criteria of the American Rheumatism Association Diagnostic and Therapeutic Criteria Committee. Arthritis Rheum. 1980;23(5):581–90.CrossRef Masi AT, Rodnan GP, Medsger TA Jr, Altman RD, D’Angelo WA, Fries JF, et al. Preliminary criteria for the classification of systemic sclerosis (scleroderma). Subcommittee for scleroderma criteria of the American Rheumatism Association Diagnostic and Therapeutic Criteria Committee. Arthritis Rheum. 1980;23(5):581–90.CrossRef
11.
go back to reference Cicchetti DV, D V. Guidelines, criteria, and rules of thumb for evaluating normed and standardized assessment instruments in psychology. Psychol Assess. 1994;6(4):284–90.CrossRef Cicchetti DV, D V. Guidelines, criteria, and rules of thumb for evaluating normed and standardized assessment instruments in psychology. Psychol Assess. 1994;6(4):284–90.CrossRef
12.
go back to reference Landis JR, Koch GG. The measurement of observer agreement for categorical data. Biometrics. 1977;33(1):159–74.CrossRef Landis JR, Koch GG. The measurement of observer agreement for categorical data. Biometrics. 1977;33(1):159–74.CrossRef
13.
go back to reference Sebastiani M, Manfredi A, Cassone G, Giuggioli D, Ghizzoni C, Ferri C. Measuring microangiopathy abnormalities in systemic sclerosis patients: the role of capillaroscopy-based scoring models. Am J Med Sci. 2014;348(4):331–6.CrossRef Sebastiani M, Manfredi A, Cassone G, Giuggioli D, Ghizzoni C, Ferri C. Measuring microangiopathy abnormalities in systemic sclerosis patients: the role of capillaroscopy-based scoring models. Am J Med Sci. 2014;348(4):331–6.CrossRef
14.
go back to reference Pavan TR, Bredemeier M, Hax V, Capobianco KG, da Silva Mendonça Chakr R, Xavier RM. Capillary loss on nailfold capillary microscopy is associated with mortality in systemic sclerosis. Clin Rheumatol. 2018;37(2):475–81.CrossRef Pavan TR, Bredemeier M, Hax V, Capobianco KG, da Silva Mendonça Chakr R, Xavier RM. Capillary loss on nailfold capillary microscopy is associated with mortality in systemic sclerosis. Clin Rheumatol. 2018;37(2):475–81.CrossRef
15.
go back to reference Ruaro B, Sulli A, Alessandri E, Pizzorni C, Ferrari G, Cutolo M, et al. Laser speckle contrast analysis: a new method to evaluate peripheral blood perfusion in systemic sclerosis patients. Ann Rheum Dis. 2014;73(6):1181–5.CrossRef Ruaro B, Sulli A, Alessandri E, Pizzorni C, Ferrari G, Cutolo M, et al. Laser speckle contrast analysis: a new method to evaluate peripheral blood perfusion in systemic sclerosis patients. Ann Rheum Dis. 2014;73(6):1181–5.CrossRef
16.
go back to reference Lüders S, Friedrich S, Ohrndorf S, Glimm A-M, Burmester G-R, Riemekasten G, et al. Detection of severe digital vasculopathy in systemic sclerosis by colour Doppler sonography is associated with digital ulcers. Rheumatology. 2017;56(11):1865–73.CrossRef Lüders S, Friedrich S, Ohrndorf S, Glimm A-M, Burmester G-R, Riemekasten G, et al. Detection of severe digital vasculopathy in systemic sclerosis by colour Doppler sonography is associated with digital ulcers. Rheumatology. 2017;56(11):1865–73.CrossRef
17.
go back to reference Sebastiani M, Cestelli V, Manfredi A, Praino E, Cannarile F, Spinella A, et al. SAT0621 Validation Study of Predictive Value of Capillaroscopic Skin Ulcer Risk Index (CSURI) in Scleroderma Patients Treated with Bosentan. Ann Rheum Dis. 2015;74(Suppl 2):886.1–886.CrossRef Sebastiani M, Cestelli V, Manfredi A, Praino E, Cannarile F, Spinella A, et al. SAT0621 Validation Study of Predictive Value of Capillaroscopic Skin Ulcer Risk Index (CSURI) in Scleroderma Patients Treated with Bosentan. Ann Rheum Dis. 2015;74(Suppl 2):886.1–886.CrossRef
19.
go back to reference Rosen RC, Cappelleri JC, Smith MD, Lipsky J, Peña BM. Development and evaluation of an abridged, 5-item version of the International Index of Erectile Function (IIEF-5) as a diagnostic tool for erectile dysfunction. Int J Impot Res. 1999;11(6):319–26.CrossRef Rosen RC, Cappelleri JC, Smith MD, Lipsky J, Peña BM. Development and evaluation of an abridged, 5-item version of the International Index of Erectile Function (IIEF-5) as a diagnostic tool for erectile dysfunction. Int J Impot Res. 1999;11(6):319–26.CrossRef
20.
go back to reference Merkel PA, Herlyn K, Martin RW, Anderson JJ, Mayes MD, Bell P, et al. Measuring disease activity and functional status in patients with scleroderma and Raynaud’s phenomenon. Arthritis Rheum. 2002;46(9):2410–20.CrossRef Merkel PA, Herlyn K, Martin RW, Anderson JJ, Mayes MD, Bell P, et al. Measuring disease activity and functional status in patients with scleroderma and Raynaud’s phenomenon. Arthritis Rheum. 2002;46(9):2410–20.CrossRef
Metadata
Title
Prospective evaluation of the capillaroscopic skin ulcer risk index in systemic sclerosis patients in clinical practice: a longitudinal, multicentre study
Authors
Ulrich A. Walker
Veronika K. Jaeger
Katharina M. Bruppacher
Rucsandra Dobrota
Lionel Arlettaz
Martin Banyai
Jörg Beron
Carlo Chizzolini
Ernst Groechenig
Rüdiger B. Mueller
François Spertini
Peter M. Villiger
Oliver Distler
Publication date
01-12-2018
Publisher
BioMed Central
Published in
Arthritis Research & Therapy / Issue 1/2018
Electronic ISSN: 1478-6362
DOI
https://doi.org/10.1186/s13075-018-1733-6

Other articles of this Issue 1/2018

Arthritis Research & Therapy 1/2018 Go to the issue
Live Webinar | 27-06-2024 | 18:00 (CEST)

Keynote webinar | Spotlight on medication adherence

Live: Thursday 27th June 2024, 18:00-19:30 (CEST)

WHO estimates that half of all patients worldwide are non-adherent to their prescribed medication. The consequences of poor adherence can be catastrophic, on both the individual and population level.

Join our expert panel to discover why you need to understand the drivers of non-adherence in your patients, and how you can optimize medication adherence in your clinics to drastically improve patient outcomes.

Prof. Kevin Dolgin
Prof. Florian Limbourg
Prof. Anoop Chauhan
Developed by: Springer Medicine
Obesity Clinical Trial Summary

At a glance: The STEP trials

A round-up of the STEP phase 3 clinical trials evaluating semaglutide for weight loss in people with overweight or obesity.

Developed by: Springer Medicine

Highlights from the ACC 2024 Congress

Year in Review: Pediatric cardiology

Watch Dr. Anne Marie Valente present the last year's highlights in pediatric and congenital heart disease in the official ACC.24 Year in Review session.

Year in Review: Pulmonary vascular disease

The last year's highlights in pulmonary vascular disease are presented by Dr. Jane Leopold in this official video from ACC.24.

Year in Review: Valvular heart disease

Watch Prof. William Zoghbi present the last year's highlights in valvular heart disease from the official ACC.24 Year in Review session.

Year in Review: Heart failure and cardiomyopathies

Watch this official video from ACC.24. Dr. Biykem Bozkurt discusses last year's major advances in heart failure and cardiomyopathies.