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Idiopathic inflammatory myopathies: state of the art on clinical practice guidelines

  • Meyer, Alain1, 2
  • Scirè, Carlo Alberto3
  • Talarico, Rosaria4
  • Alexander, Tobias5
  • Amoura, Zahir6
  • Avcin, Tadej7
  • Barsotti, Simone8, 9
  • Beretta, Lorenzo10
  • Blagojevic, Jelena11
  • Burmester, Gerd5
  • Cavazzana, Ilaria12
  • Cherrin, Patrick6
  • Damian, Laura13
  • Doria, Andrea14
  • Fonseca, João Eurico15
  • Furini, Federica16
  • Galetti, Ilaria17
  • Houssiau, Frederic18
  • Krieg, Thomas19
  • Larosa, Maddalena14
  • And 24 more
  • 1 Hôpitaux Universitaires de Strasbourg, Strasbourg, France , Strasbourg (France)
  • 2 Université de Strasbourg, Strasbourg, France , Strasbourg (France)
  • 3 University of Ferrara, Ferrara, Italy , Ferrara (Italy)
  • 4 AOU Pisana, Pisa, Italy , Pisa (Italy)
  • 5 Charité University Hospital Berlin, Berlin, Germany , Berlin (Germany)
  • 6 Hospital Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris, Paris, France , Paris (France)
  • 7 University Children's Hospital, University Medical Centre Ljubljana, Ljubljana, Slovenia , Ljubljana (Slovenia)
  • 8 University of Pisa, Pisa, Italy , Pisa (Italy)
  • 9 University of Siena, Siena, Italy , Siena (Italy)
  • 10 Referral Center for Systemic Autoimmue Diseases, Referral Center for Systemic Autoimmue DiseasesFondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico di Milano, Milan, Italy , Milan (Italy)
  • 11 University of Florence, AOUC, Florence, Italy , Florence (Italy)
  • 12 Civil Hospital, Brescia, Italy , Brescia (Italy)
  • 13 Emergency County Teaching Hospital, Cluj-Napoca, Romania , Cluj-Napoca (Romania)
  • 14 AO Padova and University of Padua, Padua, Italy , Padua (Italy)
  • 15 Hospital de Santa Maria, Centro Hospitalar Lisboa Norte, Lisbon Academic Medical Centre, Lisbon, Portugal , Lisbon (Portugal)
  • 16 Sant’Anna University Hospital, Ferrara, Italy , Ferrara (Italy)
  • 17 FESCA, Federation of European Scleroderma Associations, Milan, Italy , Milan (Italy)
  • 18 Cliniques Universitaires Saint-Luc, Université Catholique de Louvain, Louvain-la-Neuve, Belgium , Louvain-la-Neuve (Belgium)
  • 19 Universitätsklinikum Köln, Cologne, Germany , Cologne (Germany)
  • 20 Université de Lille, CHU Lille, LIRIC, INSERM, Lille, France , Lille (France)
  • 21 Centre National de Référence des Maladies Systémiques et Auto-immunes Rares Grand-Est Sud-Ouest (RESO), Hôpitaux Universitaires de Strasbourg, France, Strasbourg, France , Strasbourg (France)
  • 22 University and IRCCS Policlinico S. Matteo Foundation, Pavia, Italy , Pavia (Italy)
  • 23 Hospital de Curry Cabral, Centro Hospitalar de Lisboa Central, Lisbon, Portugal , Lisbon (Portugal)
  • 24 Hôpital Cochin, Université Paris Descartes, Paris, France , Paris (France)
  • 25 IRCCS Polyclinic Hospital San Martino, University of Genoa, Genoa, Italy , Genoa (Italy)
  • 26 Ghent University Hospital, Ghent University, Ghent, Belgium , Ghent (Belgium)
  • 27 Kerckhoff Klinik, Justus-Liebig University of Giessen, Bad Nauheim, Germany , Bad Nauheim (Germany)
  • 28 Universitätsklinikum Düsseldorf, Düsseldorf, Germany , Düsseldorf (Germany)
  • 29 Núcleo Síndrome de Sjögren, Lisbon, Portugal , Lisbon (Portugal)
Published Article
RMD Open
Publication Date
Feb 26, 2019
Suppl 1
DOI: 10.1136/rmdopen-2018-000784
PMID: 30886730
PMCID: PMC6397434
PubMed Central


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.

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