You are here

Article Text

Article menu

This article has a correction. Please see:

Download PDFPDF

Connective tissue diseases
Review
Idiopathic inflammatory myopathies: state of the art on clinical practice guidelines
  1. Alain Meyer1,2,
  2. Carlo Alberto Scirè3,
  3. Rosaria Talarico4,
  4. Tobias Alexander5,
  5. Zahir Amoura6,
  6. Tadej Avcin7,
  7. Simone Barsotti8,9,
  8. Lorenzo Beretta10,
  9. Jelena Blagojevic11,
  10. Gerd Burmester5,
  11. Ilaria Cavazzana12,
  12. Patrick Cherrin6,
  13. Laura Damian13,
  14. Andrea Doria14,
  15. João Eurico Fonseca15,
  16. Federica Furini16,
  17. Ilaria Galetti17,
  18. Frederic Houssiau18,
  19. Thomas Krieg19,
  20. Maddalena Larosa14,
  21. David Launay20,
  22. Raquel Campanilho-Marques15,
  23. Thierry Martin21,
  24. Marco Matucci-Cerinic11,
  25. Pia Moinzadeh19,
  26. Carlomaurizio Montecucco22,
  27. Maria Francisca Moraes-Fontes23,
  28. Luc Mouthon24,
  29. Rossella Neri4,
  30. Sabrina Paolino25,
  31. Yves Piette26,
  32. Simona Rednic13,
  33. Farah Tamirou18,
  34. Angela Tincani12,
  35. Natasa Toplak7,
  36. Stefano Bombardieri27,
  37. Eric Hachulla20,
  38. Ulf Mueller-Ladner28,
  39. Matthias Schneider29,
  40. Vanessa Smith26,
  41. Ana Vieira30,
  42. Maurizio Cutolo25,
  43. Marta Mosca4,8 and
  44. Lorenzo Cavagna22
  1. 1Centre National de Référence des Maladies Systémiques et Auto-immunes Rares Grand-Est Sud-Ouest (RESO), Service de humatologie, Service de physiologie, Unité d’explorations fonctionnelles musculaires, Hôpitaux Universitaires de Strasbourg, Strasbourg, France
  2. 2EA3072, Fédération de Médecine Translationelle, Université de Strasbourg, Strasbourg, France
  3. 3Section of Rheumatology, Department of Medical Sciences, University of Ferrara, Ferrara, Italy
  4. 4Rheumatology Unit, AOU Pisana, Pisa, Italy
  5. 5Department of Rheumatology and Clinical Immunology, Charité University Hospital Berlin, Berlin, Germany
  6. 6Department of Internal Medicine, Hospital Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris, Paris, France
  7. 7Department of Allergology, Rheumatology and Clinical Immunology, University Children's Hospital, University Medical Centre Ljubljana, Ljubljana, Slovenia
  8. 8Rheumatology Unit, University of Pisa, Pisa, Italy
  9. 9Department of Medical Biotechnology, University of Siena, Siena, Italy
  10. 10Referral Center for Systemic Autoimmue Diseases, Referral Center for Systemic Autoimmue DiseasesFondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico di Milano, Milan, Italy
  11. 11Department of Clinical and Experimental Medicine, Department of Geriatric Medicine, Division of Rheumatology and Scleroderma Unit, University of Florence, AOUC, Florence, Italy
  12. 12Rheumatology and Clinical Immunology Unit, Civil Hospital, Brescia, Italy
  13. 13Department of Rheumatology, Emergency County Teaching Hospital, Cluj-Napoca, Romania
  14. 14Rheumatology Unit, Department of Medicine, AO Padova and University of Padua, Padua, Italy
  15. 15Rheumatology Department, Hospital de Santa Maria, Centro Hospitalar Lisboa Norte, Lisbon Academic Medical Centre, Lisbon, Portugal
  16. 16Section of Rheumatology, Department of Medical Sciences, Sant’Anna University Hospital, Ferrara, Italy
  17. 17FESCA, Federation of European Scleroderma Associations, Milan, Italy
  18. 18Department of Rheumatology, Cliniques Universitaires Saint-Luc, Université Catholique de Louvain, Louvain-la-Neuve, Belgium
  19. 19Department of Dermatology, Universitätsklinikum Köln, Cologne, Germany
  20. 20Département de Médecine Interne et Immunologie Clinique, Centre de Référence des Maladies Systémiques et Auto-Immunes Rares du Nord-Ouest (CERAINO), Université de Lille, CHU Lille, LIRIC, INSERM, Lille, France
  21. 21Departement of Internal Medicine, Centre National de Référence des Maladies Systémiques et Auto-immunes Rares Grand-Est Sud-Ouest (RESO), Hôpitaux Universitaires de Strasbourg, France, Strasbourg, France
  22. 22Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation, Pavia, Italy
  23. 23Unidade de Doenças Auto-imunes/Medicina 7.2, Hospital de Curry Cabral, Centro Hospitalar de Lisboa Central, Lisbon, Portugal
  24. 24Service de Médecine Interne, Centre de Référence Maladies systémiques Autoimmunes Rares d’Ile de France, Assistance Publique-Hôpitaux de Paris (AP-HP), Hôpital Cochin, Université Paris Descartes, Paris, France
  25. 25Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, IRCCS Polyclinic Hospital San Martino, University of Genoa, Genoa, Italy
  26. 26Department of Rheumatology, Department of Internal Medicine, Ghent University Hospital, Ghent University, Ghent, Belgium
  27. 27University of Pisa, Pisa, Italy
  28. 28Department of Rheumatology and Clinical Immunology, Kerckhoff Klinik, Justus-Liebig University of Giessen, Bad Nauheim, Germany
  29. 29Department of Rheumatology, Universitätsklinikum Düsseldorf, Düsseldorf, Germany
  30. 30Liga Portuguesa Contra as Doenças Reumáticas, Núcleo Síndrome de Sjögren, Lisbon, Portugal
  1. Correspondence to Dr Alain Meyer; alain.meyer7{at}gmail.com

Abstract

Idiopathic inflammatory myopathies (IIMs) encompass a heterogeneous group of rare autoimmune diseases characterised by muscle weakness and inflammation, but in antisynthetase syndrome arthritis and interstitial lung disease are more frequent and often inaugurate the disease. Clinical practice guidelines (CPGs) have been proposed for IIMs, but they are sparse and heterogeneous. This work aimed at identifying: i) current available CPGs for IIMs, ii) patients ’ and clinicians’ unmet needs not covered by CPGs. It has been performed in the framework of the European Reference Network on rare and complex connective tissue and musculoskeletal diseases (ReCONNET), a network of centre of expertise and patients funded by the European Union’s Health Programme. Fourteen original CPGs were identified, notably recommending that: i) extra-muscular involvements should be assessed; ii) corticosteroids and methotrexate or azathioprine are first-line therapies of IIMs. ii) IVIG is a treatment of resistant-DM that may be also used in other resistant-IIMs; iii) physical therapy and sun protection (in DM patients) are part of the treatment; v) tumour screening for patients with DM include imaging of chest, abdomen, pelvis and breast (in woman) along with colonoscopy (in patients over 50 years); vi) disease activity and damages should be monitor using standardised and validated tools. Yet, only half of these CPGs were evidence-based. Crucial unmet needs were identified both by patients and clinicians. In particular, there was a lack of large multidisciplinary working group and of patients ’ preferences. The following fields were not or inappropriately targeted: diagnosis; management of extra-muscular involvements other than skin; co-morbidities and severe manifestations.

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/.

https://doi.org/10.1136/rmdopen-2018-000784

Statistics from Altmetric.com

    Request Permissions

    If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.

    View Full Text

    Footnotes

    • Contributors AM, SCA,TR, CM, MM and LC contributed to the conception and design of the work, acquisition of data, drafting the work and revising it critically for important intellectual content. All authors contributed to the analysis and interpretation of data, final approval of the version to be published and agreed 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), Framework Partnership Agreement number: 739531 – ERN ReCONNET. 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.

    • 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.

    • Competing interests None declared.

    • Patient consent for publication Not required.

    • Data sharing statement No additional data are available.