Observational Study

. 2019 Jan 24;21(1):35.

doi: 10.1186/s13075-019-1822-1.

Classification, categorization and essential items for digital ulcer evaluation in systemic sclerosis: a DeSScipher/European Scleroderma Trials and Research group (EUSTAR) survey

J Blagojevic¹, S Bellando-Randone², G Abignano^{3

4}, J Avouac⁵, L Cometi², L Czirják⁶, C P Denton⁷, O Distler⁸, M Frerix⁹, S Guiducci², D Huscher¹⁰, V K Jaeger¹¹, V Lóránd⁶, B Maurer⁸, S Nihtyanova⁷, G Riemekasten¹², E Siegert¹³, I H Tarner⁹, S Vettori¹⁴, U A Walker¹¹, Y Allanore⁵, U Müller-Ladner⁹, F Del Galdo³, M Matucci-Cerinic²; EUSTAR co-workers

Affiliations

¹ Department of Experimental and Clinical Medicine, University of Florence, and Department of Geriatric Medicine, Division of Rheumatology and Scleroderma Unit AOUC, Villa Monna Tessa, viale Pieraccini 18, 50139, Florence, Italy. jelena308@hotmail.com.
² Department of Experimental and Clinical Medicine, University of Florence, and Department of Geriatric Medicine, Division of Rheumatology and Scleroderma Unit AOUC, Villa Monna Tessa, viale Pieraccini 18, 50139, Florence, Italy.
³ NIHR Leeds Biomedical Research Centre, Leeds Teaching Hospitals NHS Trust and Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK.
⁴ Rheumatology Institute of Lucania (IReL), Rheumatology Department of Lucania, San Carlo Hospital of Potenza and Madonna delle Grazie Hospital of Matera, Potenza, Italy.
⁵ Department of Rheumatology, University of Paris Descartes, Paris, France.
⁶ Department of Rheumatology and Immunology, University of Pécs, Pécs, Hungary.
⁷ Department of Rheumatology, University College London, Royal Free Hospital, London, UK.
⁸ Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland.
⁹ Department of Rheumatology and Clinical Immunology, Kerckhoff-Klinik GmbH, Campus of the Justus-Liebig University Giessen, Bad Nauheim, Germany.
¹⁰ Institute of Biometry and Clinical Epidemiology, Charité - Universitätsmedizin Berlin, Corporate member of Freie Universitaet Berlin, Humboldt-Universitaet zu Berlin, and Berlin Institute of Health, Berlin, Germany.
¹¹ Department of Rheumatology, University of Basel, Basel, Switzerland.
¹² Clinic of Rheumatology and Clinical Immunology, University of Lübeck, Lübeck, Germany.
¹³ Department of Rheumatology and Clinical Immunology, Charité - Universitaetsmedizin Berlin, Corporate member of Freie Universitaet Berlin, Humboldt-Universitaet zu Berlin, and Berlin Institute of Health, Berlin, Germany.
¹⁴ Rheumatology Section, Department of Precision Medicine, University of Campania "Luigi Vanvitelli", Naples, Italy.

PMID: 30678703
PMCID: PMC6346551
DOI: 10.1186/s13075-019-1822-1

Observational Study

Classification, categorization and essential items for digital ulcer evaluation in systemic sclerosis: a DeSScipher/European Scleroderma Trials and Research group (EUSTAR) survey

J Blagojevic et al. Arthritis Res Ther. 2019.

. 2019 Jan 24;21(1):35.

doi: 10.1186/s13075-019-1822-1.

Authors

Affiliations

¹ Department of Experimental and Clinical Medicine, University of Florence, and Department of Geriatric Medicine, Division of Rheumatology and Scleroderma Unit AOUC, Villa Monna Tessa, viale Pieraccini 18, 50139, Florence, Italy. jelena308@hotmail.com.
² Department of Experimental and Clinical Medicine, University of Florence, and Department of Geriatric Medicine, Division of Rheumatology and Scleroderma Unit AOUC, Villa Monna Tessa, viale Pieraccini 18, 50139, Florence, Italy.
³ NIHR Leeds Biomedical Research Centre, Leeds Teaching Hospitals NHS Trust and Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK.
⁴ Rheumatology Institute of Lucania (IReL), Rheumatology Department of Lucania, San Carlo Hospital of Potenza and Madonna delle Grazie Hospital of Matera, Potenza, Italy.
⁵ Department of Rheumatology, University of Paris Descartes, Paris, France.
⁶ Department of Rheumatology and Immunology, University of Pécs, Pécs, Hungary.
⁷ Department of Rheumatology, University College London, Royal Free Hospital, London, UK.
⁸ Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland.
⁹ Department of Rheumatology and Clinical Immunology, Kerckhoff-Klinik GmbH, Campus of the Justus-Liebig University Giessen, Bad Nauheim, Germany.
¹⁰ Institute of Biometry and Clinical Epidemiology, Charité - Universitätsmedizin Berlin, Corporate member of Freie Universitaet Berlin, Humboldt-Universitaet zu Berlin, and Berlin Institute of Health, Berlin, Germany.
¹¹ Department of Rheumatology, University of Basel, Basel, Switzerland.
¹² Clinic of Rheumatology and Clinical Immunology, University of Lübeck, Lübeck, Germany.
¹³ Department of Rheumatology and Clinical Immunology, Charité - Universitaetsmedizin Berlin, Corporate member of Freie Universitaet Berlin, Humboldt-Universitaet zu Berlin, and Berlin Institute of Health, Berlin, Germany.
¹⁴ Rheumatology Section, Department of Precision Medicine, University of Campania "Luigi Vanvitelli", Naples, Italy.

PMID: 30678703
PMCID: PMC6346551
DOI: 10.1186/s13075-019-1822-1

Abstract

Background: A consensus on digital ulcer (DU) definition in systemic sclerosis (SSc) has been recently reached (Suliman et al., J Scleroderma Relat Disord 2:115-20, 2017), while for their evaluation, classification and categorisation, it is still missing. The aims of this study were to identify a set of essential items for digital ulcer (DU) evaluation, to assess if the existing DU classification was useful and feasible in clinical practice and to investigate if the new categorisation was preferred to the simple distinction of DU in recurrent and not recurrent, in patients with systemic sclerosis (SSc).

Methods: DeSScipher is the largest European multicentre study on SSc. It consists of five observational trials (OTs), and one of them, OT1, is focused on DU management. The DeSScipher OT1 items on DU that reached ≥ 60% of completion rate were administered to EUSTAR (European Scleroderma Trials and Research group) centres via online survey. Questions about feasibility and usefulness of the existing DU classification (DU due to digital pitting scars, to loss of tissue, derived from calcinosis and gangrene) and newly proposed categorisation (episodic, recurrent and chronic) were also asked.

Results: A total of 84/148 (56.8%) EUSTAR centres completed the questionnaire. DeSScipher items scored by ≥ 70% of the participants as essential and feasible for DU evaluation were the number of DU defined as a loss of tissue (level of agreement 92%), recurrent DU (84%) and number of new DU (74%). For 65% of the centres, the proposed classification of DU was considered useful and feasible in clinical practice. Moreover, 80% of the centres preferred the categorisation of DU in episodic, recurrent and chronic to simple distinction in recurrent/not recurrent DU.

Conclusions: For clinical practice, EUSTAR centres identified only three essential items for DU evaluation and considered the proposed classification and categorisation as useful and feasible. The set of items needs to be validated while further implementation of DU classification and categorisation is warranted.

Trial registration: Observational trial on DU (OT1) is one of the five trials of the DeSScipher project (ClinicalTrials.gov; OT1 Identifier: NCT01836263 , posted on April 19, 2013).

Keywords: Categorisation; Classification; Digital ulcers; Essential items; Systemic sclerosis.

PubMed Disclaimer

Conflict of interest statement

Ethics approval and consent to participate

Ethical approval of DeSScipher OT1 has been obtained from all participating centres’ local ethics committees (project coordinator’s ethics board: Ethics Review board of the Justus-Liebig University Giessen, Germany, approval no 02/13; partners centres ethics review boards: University of Zurich, Switzerland; University of Paris, France; University of Florence Italy; The Second University of Naples, Italy; University of Basel, Switzerland; University College of London, United Kingdom; University of Berlin Charité, Germany; University of Pécs, Hungary; University of Leeds, United Kingdom; and contributors centres ethics boards (additional 21 centres)). Each patient signed a written informed consent form.

Consent for publication

Not applicable.

Competing interests

The authors report personal fees and non-financial support by the European Community’s Framework Program 7 [FP7-HEALTH-2012.2.4.4-2 Observational trials in rare diseases]; grant agreement no. 305495.

OD: Consultancies, speaking fees, and honoraria < $10,000: AnaMar, Bayer, Boehringer Ingelheim, Catenion, CLS Behring, ChemomAb, Roche, GSK, Inventiva, Italfarmaco, Lilly, medac, Medscape, Mitsubishi Tanabe Pharma, MSD, Novartis, Pfizer, Sanofi, UCB Consultancies, speaking fees, and honoraria > $10,000: Bayer, Böhringer. Patents, licenses, or licensing fees: Patent mir-29 for the treatment of systemic sclerosis licensed

CPD: Consultancies, speaking fees, and honoraria < $10,000: Actelion, Bayer, Boehringer Ingelheim, Inventiva, Sanofi-Aventis, CSL Behring

SV: Consultancies, speaking fees, and honoraria < $10,000: Thermofisher, Abbvie, Boehringer-Ingelheim

YA: Consultancies, speaking fees, and honoraria < $10,000: Actelion, Boehringer, Roche, Sanofi, Inventiva, Medac, Bayer, BMS, Pfizer

MMC: Consultancies, speaking fees, and honoraria < $10,000: Actelion, BMS, Pfizer

Publisher’s Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Figures

**Fig. 1**
Pure DU due to loss of tissue not dependent from digital pitting scar or calcinosis

See this image and copyright information in PMC

References

1. 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;2:137–152. doi: 10.5301/jsrd.5000249. - DOI
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1. Matucci-Cerinic M, Krieg T, Guillevin L, Schwierin B, Rosenberg D, Cornelisse P, et al. Elucidating the burden of recurrent and chronic digital ulcers in systemic sclerosis: long-term results from the DUO Registry. Ann Rheum Dis. 2016;75:1770–1776. doi: 10.1136/annrheumdis-2015-208121. - DOI - PMC - PubMed
1. Mihai C, Landewé R, van der Heijde D, Walker UA, Constantin PI, Gherghe AM, et al. Digital ulcers predict a worse disease course in patients with systemic sclerosis. Ann Rheum Dis. 2016;75:681–686. doi: 10.1136/annrheumdis-2014-205897. - DOI - PubMed
1. Jaeger VK, Distler O, Maurer B, Czirják L, Lóránd V, Valentini G, et al. Functional disability and its predictors in systemic sclerosis: a study from the DeSScipher project within the EUSTAR group. Rheumatology (Oxford) 2018;57:441–450. doi: 10.1093/rheumatology/kex182. - DOI - PubMed

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Classification, categorization and essential items for digital ulcer evaluation in systemic sclerosis: a DeSScipher/European Scleroderma Trials and Research group (EUSTAR) survey

Affiliations

Classification, categorization and essential items for digital ulcer evaluation in systemic sclerosis: a DeSScipher/European Scleroderma Trials and Research group (EUSTAR) survey

Authors

Affiliations

Abstract

Conflict of interest statement

Ethics approval and consent to participate

Consent for publication

Competing interests

Publisher’s Note

Figures

References

Publication types

MeSH terms

Substances

Associated data

Grants and funding

LinkOut - more resources

Full Text Sources

Medical

Miscellaneous