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Use of vasoactive/vasodilating drugs for systemic sclerosis (SSc)-related digital ulcers (DUs) in expert tertiary centres: results from the analysis of the observational real-life DeSScipher study

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Abstract

Introduction

DeSScipher is the first European multicentre study on management of systemic sclerosis (SSc), and its observational trial 1 (OT1) evaluated the efficacy of different drugs for digital ulcer (DU) prevention and healing. The aim of this study was to assess current use of vasoactive/vasodilating agents for SSc-related DU in the expert centres by analysing the baseline data of the DeSScipher OT1.

Method

Baseline characteristics of patients enrolled in the OT1 and data regarding DU were analysed.

Results

The most commonly used drugs, in both patients with and without DU, were calcium channel blockers (CCBs) (71.6%), followed by intravenous iloprost (20.8%), endothelin receptor antagonists (ERAs) (20.4%) and phosphodiesterase 5 (PDE-5) inhibitors (16.5%). Of patients, 32.6% with DU and 12.8% without DU received two drugs (p < 0.001), while 11.5% with DU and 1.9% without DU were treated with a combination of three or more agents (p < 0.001). Sixty-five percent of the patients with recurrent DU were treated with bosentan and/or sildenafil. However, 64 out of 277 patients with current DU (23.1%) and 101 (23.6%) patients with recurrent DU were on CCBs alone.

Conclusions

Our study shows that CCBs are still the most commonly used agents for DU management in SSc. The proportion of patients on combination therapy was low, even in patients with recurrent DU: almost one out of four patients with current and recurrent DU was on CCBs alone. Prospective analysis is planned to investigate the efficacy of different drugs/drug combinations on DU healing and prevention.

Key Points

• The analysis of DeSScipher, the first European multicentre study on management of SSc, has shown that the most commonly used vasoactive/vasodilating drugs for DU were CCBs, followed by intravenous Iloprost, ERAs and PDE-5 inhibitors.

• More than half of the patients with recurrent DU received bosentan and/or sildenafil.

• However, the proportion of patients on combination therapy of more than one vasoactive/vasodilating drug was low and almost one out of four patients with current and recurrent DU was on CCBs alone.

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Funding

This study, as part of the DeSScipher project, was supported by the European Community’s Framework Programme 7 (FP7-HEALTH-2012.2.4.4-2 Observational trials in rare diseases; grant agreement no. 305495). The authors report personal fees and non-financial support (to LV) from the European Union Seventh Framework Program 7 (FP7-HEALTH-2012.2.4.4-2 Observational trials in rare diseases]; grant agreement no. 305495).

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Authors and Affiliations

  1. Department of Experimental and Clinical Medicine, Department of Geriatric Medicine, Division of Rheumatology and Scleroderma Unit AOUC, University of Florence, Florence, Italy

    Jelena Blagojevic, L. Cometi, S. Bellando-Randone, S. Guiducci, C. Bruni & M. Matucci-Cerinic

  2. Rheumatology Institute of Lucania (IReL), Rheumatology Department of Lucania, San Carlo Hospital of Potenza and Madonna delle Grazie Hospital of Matera, Potenza, Italy

    G. Abignano

  3. NIHR Leeds Biomedical Research Centre, Leeds Teaching Hospitals NHS Trust and Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK

    G. Abignano & F. Del Galdo

  4. Department of Rheumatology, University of Paris Descartes, Paris, France

    J. Avouac & Y. Allanore

  5. Department of Rheumatology and Clinical Immunology, Kerckhoff Klinik GmbH, Campus of the Justus-Liebig University Giessen, Bad Nauheim, Germany

    M. Frerix, I. H. Tarner & U. Müller-Ladner

  6. 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

    D. Huscher

  7. Department of Rheumatology, University of Basel, Basel, Switzerland

    V. K. Jaeger & U. A. Walker

  8. Department of Rheumatology and Immunology, University of Pécs, Pecs, Hungary

    V. Lóránd & L. Czirják

  9. Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland

    B. Maurer & O. Distler

  10. Department of Rheumatology, University College London, Royal Free Hospital, London, UK

    S. Nihtyanova & C. P. Denton

  11. Clinic of Rheumatology and Clinical Immunology, University of Lübeck, Lubeck, Germany

    G. Riemekasten

  12. 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

    E. Siegert

  13. Rheumatology Section, Department of Precision Medicine, University of Campania “Luigi Vanvitelli”, Naples, Italy

    S. Vettori

  14. Department of Experimental and Clinical Medicine, Department of Emergency, Division of Medicine IV AOUC, University of Florence, Florence, Italy

    A. Moggi-Pignone

Authors
  1. Jelena Blagojevic
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  2. G. Abignano
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  3. J. Avouac
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  4. L. Cometi
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  7. S. Guiducci
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  8. C. Bruni
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  13. S. Nihtyanova
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  14. G. Riemekasten
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  16. I. H. Tarner
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  18. U. A. Walker
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  19. L. Czirják
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  20. C. P. Denton
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  21. O. Distler
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  25. M. Matucci-Cerinic
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  26. F. Del Galdo
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Correspondence to Jelena Blagojevic.

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Ethical approval had been obtained from all participating centres’ local ethics committees, according to the declaration of Helsinki and its later amendments. Each patient signed a written informed consent form.

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Blagojevic, J., Abignano, G., Avouac, J. et al. Use of vasoactive/vasodilating drugs for systemic sclerosis (SSc)-related digital ulcers (DUs) in expert tertiary centres: results from the analysis of the observational real-life DeSScipher study. Clin Rheumatol 39, 27–36 (2020). https://doi.org/10.1007/s10067-019-04564-8

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