. 2018 Oct 18;4(Suppl 1):e000782.

doi: 10.1136/rmdopen-2018-000782. eCollection 2018.

Systemic sclerosis: state of the art on clinical practice guidelines

Vanessa Smith^{1

2}, Carlo Alberto Scirè^{3

4}, Rosaria Talarico⁵, Paolo Airo⁶, Tobias Alexander⁷, Yannick Allanore^{8

9}, Cosimo Bruni^{10

11}, Veronica Codullo^{12

13}, Virgil Dalm¹⁴, Jeska De Vries-Bouwstra¹⁵, Alessandra Della Rossa⁵, Oliver Distler¹⁶, Ilaria Galetti¹⁷, David Launay^{18

19}, Gemma Lepri^{10

11}, Alexis Mathian²⁰, Luc Mouthon^{8

9}, Barbara Ruaro²¹, Alberto Sulli²¹, Angela Tincani⁶, Els Vandecasteele^{2

22}, Amber Vanhaecke^{1

2}, Marie Vanthuyne^{23

24}, Frank Van den Hoogen^{25

26}, Ronald Van Vollenhoven²⁷, Alexandre E Voskuyl^{28

29}, Elisabetta Zanatta³⁰, Stefano Bombardieri³¹, Gerd Burmester³², Fonseca João Eurico^{33

34}, Charissa Frank³⁵, Eric Hachulla^{18

19}, Frederic Houssiau^{23

24}, Ulf Mueller-Ladner³⁶, Matthias Schneider³⁷, Jacob M van Laar³⁸, Ana Vieira³⁹, Maurizio Cutolo²¹, Marta Mosca⁴⁰, Marco Matucci-Cerinic^{10

11}

Affiliations

¹ Department of Rheumatology, Ghent University Hospital, Ghent, Belgium.
² Department of Internal Medicine, Ghent University, Ghent, Belgium.
³ Section of Rheumatology, Department of Medical Sciences, University of Ferrara, Ferrara, Italy.
⁴ Epidemiology Unit, Italian Society for Rheumatology (SIR), Milan, Italy.
⁵ Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy.
⁶ Rheumatology and Clinical Immunology Unit, Azienda Ospedaliera Spedali Civili di Brescia, Brescia, Italy.
⁷ Department of Rheumatology and Clinical Immunology Unit, Charité University Hospital Berlin, Berlin, Germany.
⁸ Service de Médicine Interne, Université Paris Descartes, Paris, France.
⁹ Centre de Référence Maladies systémiques Autoimmunes Rares d'Ile de France, Assistance Publique-Hôpitaux de Paris (AP-HP), Hôpital Cochin, Paris, France.
¹⁰ Department of Clinical and Experimental Medicine, University of Florence, Florence, Italy.
¹¹ Division of Rheumatology and Scleroderma Unit, AOU Careggi, Florence, Italy.
¹² Department of Rheumatology, University of Pavia, Pavia, Italy.
¹³ Department of Rheumatology, IRCCS Policlinico San Matteo, Pavia, Italy.
¹⁴ Department of Internal Medicine and Department of Immunology, Erasmus Medical Center, Rotterdam, The Netherlands.
¹⁵ Department of Rheumatology, Leiden University Medical Center, Leiden, The Netherlands.
¹⁶ Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland.
¹⁷ Federation of European Scleroderma Associations (FESCA), Brussels, Belgium.
¹⁸ Département de Médecine Interne et Immunologie Clinique, Université de Lille, Lille, France.
¹⁹ Centre de Référence des Maladies Systémiques et Auto-Immunes Rares du Nord-Ouest (CERAINO), LIRIC, INSERM, CHU Lille, Lille, France.
²⁰ Department of Internal Medicine, Hospital Pitié-Salpêtrière, Paris, France.
²¹ Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, IRCCS San Martino Polyclinic Hospital, University of Genoa, Genoa, Italy.
²² Department of Cardiology, Ghent University Hospital, Ghent, Belgium.
²³ Department of Rheumatology, Université Catholique de Louvain, Louvain-la-Neuve, Belgium.
²⁴ Department of Rheumatology, Cliniques Universitaires Saint-Luc, Louvain-la-Neuve, Belgium.
²⁵ Department of Rheumatology, Radboud University Medical Center Nijmegen, Nijmegen, The Netherlands.
²⁶ Department of Rheumatology, Sint Maartenskliniek, Nijmegen, The Netherlands.
²⁷ Clinical Immunology & Rheumatology, Amsterdam Rheumatology & Immunology Center, Academic Medical Center/University of Amsterdam, Amsterdam, The Netherlands.
²⁸ Department of Rheumatology, Amsterdam UMC, Amsterdam, The Netherlands.
²⁹ Amsterdam Infection & Immunity Institute, Vrije Universiteit Amsterdam, Amsterdam, The Netherlands.
³⁰ Division of Rheumatology, Department of Medicine, University of Padova, Padova, Italy.
³¹ Rheumatology Unit, Department of Clinical & Experimental Medicine, University of Pisa, Pisa, Italy.
³² Department of Rheumatology and Clinical Immunology, Charité University Hospital Berlin, Berlin, Germany.
³³ Department of Rheumatology, Hospital de Santa Maria, Centro Hospitalar Lisboa Norte, Lisbon, Portugal.
³⁴ Instituto de Medicina Molecular, Faculdade de Medicina, Universidade de Lisboa, Lisbon, Portugal.
³⁵ Flemish Patient Organization of Hereditary Collagen Disorders in Belgium, Koersel, Belgium.
³⁶ Department of Rheumatology and Clinical Immunology, Kerckhoff Klinik, Justus-Liebig University of Giessen, Giessen, Germany.
³⁷ Institute for Rheumatology, Hiller Research Unit for Rheumatology, Medical Faculty, Heinrich-Heine University Düsseldorf, Düsseldorf, Germany.
³⁸ Department of Rheumatology and Clinical Immunology, University Medical Center Utrecht, Utrecht, The Netherlands.
³⁹ Núcleo Síndrome de Sjögren of Liga Portuguesa Contra as Doenças Reumáticas (LPCDR, Portuguese League Against Rheumatic Diseases), Lisbon, Portugal.
⁴⁰ Rheumatology Unit, Department of Internal Medicine, University of Pisa, Pisa, Italy.

PMID: 30402270
PMCID: PMC6203100
DOI: 10.1136/rmdopen-2018-000782

Systemic sclerosis: state of the art on clinical practice guidelines

Vanessa Smith et al. RMD Open. 2018.

. 2018 Oct 18;4(Suppl 1):e000782.

doi: 10.1136/rmdopen-2018-000782. eCollection 2018.

Authors

Affiliations

¹ Department of Rheumatology, Ghent University Hospital, Ghent, Belgium.
² Department of Internal Medicine, Ghent University, Ghent, Belgium.
³ Section of Rheumatology, Department of Medical Sciences, University of Ferrara, Ferrara, Italy.
⁴ Epidemiology Unit, Italian Society for Rheumatology (SIR), Milan, Italy.
⁵ Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy.
⁶ Rheumatology and Clinical Immunology Unit, Azienda Ospedaliera Spedali Civili di Brescia, Brescia, Italy.
⁷ Department of Rheumatology and Clinical Immunology Unit, Charité University Hospital Berlin, Berlin, Germany.
⁸ Service de Médicine Interne, Université Paris Descartes, Paris, France.
⁹ Centre de Référence Maladies systémiques Autoimmunes Rares d'Ile de France, Assistance Publique-Hôpitaux de Paris (AP-HP), Hôpital Cochin, Paris, France.
¹⁰ Department of Clinical and Experimental Medicine, University of Florence, Florence, Italy.
¹¹ Division of Rheumatology and Scleroderma Unit, AOU Careggi, Florence, Italy.
¹² Department of Rheumatology, University of Pavia, Pavia, Italy.
¹³ Department of Rheumatology, IRCCS Policlinico San Matteo, Pavia, Italy.
¹⁴ Department of Internal Medicine and Department of Immunology, Erasmus Medical Center, Rotterdam, The Netherlands.
¹⁵ Department of Rheumatology, Leiden University Medical Center, Leiden, The Netherlands.
¹⁶ Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland.
¹⁷ Federation of European Scleroderma Associations (FESCA), Brussels, Belgium.
¹⁸ Département de Médecine Interne et Immunologie Clinique, Université de Lille, Lille, France.
¹⁹ Centre de Référence des Maladies Systémiques et Auto-Immunes Rares du Nord-Ouest (CERAINO), LIRIC, INSERM, CHU Lille, Lille, France.
²⁰ Department of Internal Medicine, Hospital Pitié-Salpêtrière, Paris, France.
²¹ Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, IRCCS San Martino Polyclinic Hospital, University of Genoa, Genoa, Italy.
²² Department of Cardiology, Ghent University Hospital, Ghent, Belgium.
²³ Department of Rheumatology, Université Catholique de Louvain, Louvain-la-Neuve, Belgium.
²⁴ Department of Rheumatology, Cliniques Universitaires Saint-Luc, Louvain-la-Neuve, Belgium.
²⁵ Department of Rheumatology, Radboud University Medical Center Nijmegen, Nijmegen, The Netherlands.
²⁶ Department of Rheumatology, Sint Maartenskliniek, Nijmegen, The Netherlands.
²⁷ Clinical Immunology & Rheumatology, Amsterdam Rheumatology & Immunology Center, Academic Medical Center/University of Amsterdam, Amsterdam, The Netherlands.
²⁸ Department of Rheumatology, Amsterdam UMC, Amsterdam, The Netherlands.
²⁹ Amsterdam Infection & Immunity Institute, Vrije Universiteit Amsterdam, Amsterdam, The Netherlands.
³⁰ Division of Rheumatology, Department of Medicine, University of Padova, Padova, Italy.
³¹ Rheumatology Unit, Department of Clinical & Experimental Medicine, University of Pisa, Pisa, Italy.
³² Department of Rheumatology and Clinical Immunology, Charité University Hospital Berlin, Berlin, Germany.
³³ Department of Rheumatology, Hospital de Santa Maria, Centro Hospitalar Lisboa Norte, Lisbon, Portugal.
³⁴ Instituto de Medicina Molecular, Faculdade de Medicina, Universidade de Lisboa, Lisbon, Portugal.
³⁵ Flemish Patient Organization of Hereditary Collagen Disorders in Belgium, Koersel, Belgium.
³⁶ Department of Rheumatology and Clinical Immunology, Kerckhoff Klinik, Justus-Liebig University of Giessen, Giessen, Germany.
³⁷ Institute for Rheumatology, Hiller Research Unit for Rheumatology, Medical Faculty, Heinrich-Heine University Düsseldorf, Düsseldorf, Germany.
³⁸ Department of Rheumatology and Clinical Immunology, University Medical Center Utrecht, Utrecht, The Netherlands.
³⁹ Núcleo Síndrome de Sjögren of Liga Portuguesa Contra as Doenças Reumáticas (LPCDR, Portuguese League Against Rheumatic Diseases), Lisbon, Portugal.
⁴⁰ Rheumatology Unit, Department of Internal Medicine, University of Pisa, Pisa, Italy.

PMID: 30402270
PMCID: PMC6203100
DOI: 10.1136/rmdopen-2018-000782

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.

Keywords: ERN ReCONNET; European reference networks; clinical practice guidelines; nailfold videocapillaroscopy; systemic sclerosis; unmet needs.

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Conflict of interest statement

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).

Figures

**Figure 1**
Flow chart of the screening process. ERN ReCONNET, European Reference Network Rare and Complex Connective Tissue and Musculoskeletal Diseases; DC, disease coordinators; HCP, healthcare providers; HSCT, haematopoietic stem cell transplantation; ILD, interstitial lung disease; JC, Junior Coordinator; PAH, pulmonary arterial hypertension; SC, Senior Coordinator; SSc, systemic sclerosis.

See this image and copyright information in PMC

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Systemic sclerosis: state of the art on clinical practice guidelines

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Systemic sclerosis: state of the art on clinical practice guidelines

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