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Review
Systemic sclerosis: state of the art on clinical practice guidelines
  1. Vanessa Smith1,2,
  2. Carlo Alberto Scirè3,4,
  3. Rosaria Talarico5,
  4. Paolo Airo6,
  5. Tobias Alexander7,
  6. Yannick Allanore8,9,
  7. Cosimo Bruni10,11,
  8. Veronica Codullo12,13,
  9. Virgil Dalm14,
  10. Jeska De Vries-Bouwstra15,
  11. Alessandra Della Rossa5,
  12. Oliver Distler16,
  13. Ilaria Galetti17,
  14. David Launay18,19,
  15. Gemma Lepri10,11,
  16. Alexis Mathian20,
  17. Luc Mouthon8,9,
  18. Barbara Ruaro21,
  19. Alberto Sulli21,
  20. Angela Tincani6,
  21. Els Vandecasteele2,22,
  22. Amber Vanhaecke1,2,
  23. Marie Vanthuyne23,24,
  24. Frank Van den Hoogen25,26,
  25. Ronald Van Vollenhoven27,
  26. Alexandre E Voskuyl28,29,
  27. Elisabetta Zanatta30,
  28. Stefano Bombardieri31,
  29. Gerd Burmester32,
  30. João Eurico Fonseca33,34,
  31. Charissa Frank35,
  32. Eric Hachulla18,19,
  33. Frederic Houssiau23,24,
  34. Ulf Mueller-Ladner36,
  35. Matthias Schneider37,
  36. Jacob M van Laar38,
  37. Ana Vieira39,
  38. Maurizio Cutolo21,
  39. Marta Mosca40 and
  40. Marco Matucci-Cerinic10,11
  1. 1 Department of Rheumatology, Ghent University Hospital, Ghent, Belgium
  2. 2 Department of Internal Medicine, Ghent University, Ghent, Belgium
  3. 3 Section of Rheumatology, Department of Medical Sciences, University of Ferrara, Ferrara, Italy
  4. 4 Epidemiology Unit, Italian Society for Rheumatology (SIR), Milan, Italy
  5. 5 Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy
  6. 6 Rheumatology and Clinical Immunology Unit, Azienda Ospedaliera Spedali Civili di Brescia, Brescia, Italy
  7. 7 Department of Rheumatology and Clinical Immunology Unit, Charité University Hospital Berlin, Berlin, Germany
  8. 8 Service de Médicine Interne, Université Paris Descartes, Paris, France
  9. 9 Centre de Référence Maladies systémiques Autoimmunes Rares d’Ile de France, Assistance Publique-Hôpitaux de Paris (AP-HP), Hôpital Cochin, Paris, France
  10. 10 Department of Clinical and Experimental Medicine, University of Florence, Florence, Italy
  11. 11 Division of Rheumatology and Scleroderma Unit, AOU Careggi, Florence, Italy
  12. 12 Department of Rheumatology, University of Pavia, Pavia, Italy
  13. 13 Department of Rheumatology, IRCCS Policlinico San Matteo, Pavia, Italy
  14. 14 Department of Internal Medicine and Department of Immunology, Erasmus Medical Center, Rotterdam, The Netherlands
  15. 15 Department of Rheumatology, Leiden University Medical Center, Leiden, The Netherlands
  16. 16 Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland
  17. 17 Federation of European Scleroderma Associations (FESCA), Brussels, Belgium
  18. 18 Département de Médecine Interne et Immunologie Clinique, Université de Lille, Lille, France
  19. 19 Centre de Référence des Maladies Systémiques et Auto-Immunes Rares du Nord-Ouest (CERAINO), LIRIC, INSERM, CHU Lille, Lille, France
  20. 20 Department of Internal Medicine, Hospital Pitié-Salpêtrière, Paris, France
  21. 21 Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, IRCCS San Martino Polyclinic Hospital, University of Genoa, Genoa, Italy
  22. 22 Department of Cardiology, Ghent University Hospital, Ghent, Belgium
  23. 23 Department of Rheumatology, Université Catholique de Louvain, Louvain-la-Neuve, Belgium
  24. 24 Department of Rheumatology, Cliniques Universitaires Saint-Luc, Louvain-la-Neuve, Belgium
  25. 25 Department of Rheumatology, Radboud University Medical Center Nijmegen, Nijmegen, The Netherlands
  26. 26 Department of Rheumatology, Sint Maartenskliniek, Nijmegen, The Netherlands
  27. 27 Clinical Immunology & Rheumatology, Amsterdam Rheumatology & Immunology Center, Academic Medical Center/University of Amsterdam, Amsterdam, The Netherlands
  28. 28 Department of Rheumatology, Amsterdam UMC, Amsterdam, The Netherlands
  29. 29 Amsterdam Infection & Immunity Institute, Vrije Universiteit Amsterdam, Amsterdam, The Netherlands
  30. 30 Division of Rheumatology, Department of Medicine, University of Padova, Padova, Italy
  31. 31 Rheumatology Unit, Department of Clinical & Experimental Medicine, University of Pisa, Pisa, Italy
  32. 32 Department of Rheumatology and Clinical Immunology, Charité University Hospital Berlin, Berlin, Germany
  33. 33 Department of Rheumatology, Hospital de Santa Maria, Centro Hospitalar Lisboa Norte, Lisbon, Portugal
  34. 34 Instituto de Medicina Molecular, Faculdade de Medicina, Universidade de Lisboa, Lisbon, Portugal
  35. 35 Flemish Patient Organization of Hereditary Collagen Disorders in Belgium, Koersel, Belgium
  36. 36 Department of Rheumatology and Clinical Immunology, Kerckhoff Klinik, Justus-Liebig University of Giessen, Giessen, Germany
  37. 37 Institute for Rheumatology, Hiller Research Unit for Rheumatology, Medical Faculty, Heinrich-Heine University Düsseldorf, Düsseldorf, Germany
  38. 38 Department of Rheumatology and Clinical Immunology, University Medical Center Utrecht, Utrecht, The Netherlands
  39. 39 Núcleo Síndrome de Sjögren of Liga Portuguesa Contra as Doenças Reumáticas (LPCDR, Portuguese League Against Rheumatic Diseases), Lisbon, Portugal
  40. 40 Rheumatology Unit, Department of Internal Medicine, University of Pisa, Pisa, Italy
  1. Correspondence to Dr Vanessa Smith; vanessa.smith{at}ugent.be

Abstract

Systemic sclerosis (SSc) is an orphan disease characterised by autoimmunity, fibrosis of the skin and internal organs, and vasculopathy. SSc may be associated with high morbidity and mortality. In this narrative review we summarise the results of a systematic literature research, which was performed as part of the European Reference Network on Rare and Complex Connective Tissue and Musculoskeletal Diseases project, aimed at evaluating existing clinical practice guidelines or recommendations. Only in the domains ‘Vascular & Ulcers’ (ie, non-pharmacological approach to digital ulcer), ‘PAH’ (ie, screening and treatment), ‘Treatment’ and ‘Juveniles’ (ie, evaluation of juveniles with Raynaud’s phenomenon) evidence-based and consensus-based guidelines could be included. Hence there is a preponderance of unmet needs in SSc referring to the diagnosis and (non-)pharmacological treatment of several SSc-specific complications. Patients with SSc experience significant uncertainty concerning SSc-related taxonomy, management (both pharmacological and non-pharmacological) and education. Day-to-day impact of the disease (loss of self-esteem, fatigue, sexual dysfunction, and occupational, nutritional and relational problems) is underestimated and needs evaluation.

  • clinical practice guidelines
  • ERN ReCONNET
  • European reference networks
  • systemic sclerosis
  • nailfold videocapillaroscopy
  • unmet needs

This is an open access article distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited, appropriate credit is given, any changes made indicated, and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0

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Footnotes

  • Contributors VS, CAS, RT: substantial contributions to the conception and design of the work, the acquisition, analysis and interpretation of data; drafting the work and revising it critically for important intellectual content; final approval of the version to be published; agreement to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. PA, TA, YA, CB, VC, VD, JDV-B, ADR, OD, IG, DL, GL, AM, LM, BR, AS, AT, EV, AmV, MV, FVdH, RVV, AEZ, EZ: substantial contributions to the analysis and interpretation of data; final approval of the version to be published; agreement to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. SB, MC, MM, MM-C: substantial contributions to the conception and design of the work, the acquisition, analysis and interpretation of data; drafting the work and revising it critically for important intellectual content; final approval of the version to be published; agreement to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. GB, FJE, CF, EH, FH, UM-L, MS, JMvL, AnV: revising the work critically for important intellectual content; final approval of the version to be published; agreement to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.

  • Funding This publication was funded by the European Union’s Health Programme (2014-2020).

  • Disclaimer ERN ReCONNET is one of the 24 European Reference Networks (ERNs) approved by the ERN Board of Member States. The ERNs are co-funded by the European Commission. The content of this publication represents the views of the authors only and it is their sole responsibility; it cannot be considered to reflect the views of the European Commission and/or the Consumers, Health, Agriculture and Food Executive Agency (CHAFEA) or any other body of the European Union. The European Commission and the Agency do not accept any responsibility for use that may be made of the information it contains.

  • Conflicts of interest VS, None to declare. CAS, None to declare. RT, None to declare. PA, None to declare. TA, None to declare. YA, consulted for Actelion, Bayer, Roche/Genentech, Inventiva, Medac, Pfizer, Sanofi, Servier, and UCB; and has received research grants from Bristol-Myers Squibb, Roche/Genentech, Inventiva, Pfizer, Sanofi. CB, None to declare. VC, None to declare. VD, None to declare. JVB, None to declare. ADS, None to declare. OD, had consultancy relationship and/or has received research funding from Actelion, AnaMar, Bayer, Boehringer Ingelheim, Catenion, CSL Behring, ChemomAb, Roche,GSK, Inventiva, Italfarmaco, Lilly, medac, Medscape, Mitsubishi Tanabe Pharma, MSD, Novartis, Pfizer, Sanofi, and UCB in the area of potential treatments ofscleroderma and its complications. In addition, Prof. Distler has a patent mir-29 for the treatment of systemic sclerosis licensed. The real or perceived potential conflicts listed above are accurately stated. IG, None to declare. DL, None to declare. GL, None to declare. AM, None to declare. LM, None to declare. BR, None to declare. AS, None to declare. AT, None to declare. EV, None to declare. AV, None to declare. MV, None to declare. FVH, None to declare. RVV, consulted for AbbVie, AstraZeneca, Biogen, Biotest, BMS, Celgene, Gilead, GSK, Janssen, Lilly, Novartis, Pfizer, UCB; and received research support and grants from AbbVie, BMS, GSK, Pfizer, UCB. AV None to declare. EZ, None to declare. SB, None to declare. GB, None to declare. FJE, None to declare. CF, None to declare. EH, None to declare. FH, None to declare. UML, None to declare. MS, None to declare. JML, None to declare. AV, None to declare. MC, None to declare. MM, None to declare. MMC, has consultancy relationship and/or has received research funding for Actelion, BMS, Celgene, Chemomab, CSL Behring, Eli Lilly and Pfizer; and is a member of the college of emeritus presidents of the Italian Society of Rheumatology (SIR).

  • Patient consent Not required.

  • Provenance and peer review Commissioned; externally peer reviewed.

  • Data sharing statement There are no additional unpublished data from the study.

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