Format

Send to

Choose Destination
RMD Open. 2019 Feb 26;4(Suppl 1):e000784. doi: 10.1136/rmdopen-2018-000784. eCollection 2018.

Idiopathic inflammatory myopathies: state of the art on clinical practice guidelines [corrected].

Author information

1
Centre 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
EA3072, Fédération de Médecine Translationelle, Université de Strasbourg, Strasbourg, France.
3
Section of Rheumatology, Department of Medical Sciences, University of Ferrara, Ferrara, Italy.
4
Rheumatology Unit, AOU Pisana, Pisa, Italy.
5
Department of Rheumatology and Clinical Immunology, Charité University Hospital Berlin, Berlin, Germany.
6
Department of Internal Medicine, Hospital Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris, Paris, France.
7
Department of Allergology, Rheumatology and Clinical Immunology, University Children's Hospital, University Medical Centre Ljubljana, Ljubljana, Slovenia.
8
Rheumatology Unit, University of Pisa, Pisa, Italy.
9
Department of Medical Biotechnology, University of Siena, 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.
11
Department of Clinical and Experimental Medicine, Department of Geriatric Medicine, Division of Rheumatology and Scleroderma Unit, University of Florence, AOUC, Florence, Italy.
12
Rheumatology and Clinical Immunology Unit, Civil Hospital, Brescia, Italy.
13
Department of Rheumatology, Emergency County Teaching Hospital, Cluj-Napoca, Romania.
14
Rheumatology Unit, Department of Medicine, AO Padova and University of Padua, Padua, Italy.
15
Rheumatology Department, Hospital de Santa Maria, Centro Hospitalar Lisboa Norte, Lisbon Academic Medical Centre, Lisbon, Portugal.
16
Section of Rheumatology, Department of Medical Sciences, Sant'Anna University Hospital, Ferrara, Italy.
17
FESCA, Federation of European Scleroderma Associations, Milan, Italy.
18
Department of Rheumatology, Cliniques Universitaires Saint-Luc, Université Catholique de Louvain, Louvain-la-Neuve, Belgium.
19
Department of Dermatology, Universitätsklinikum Köln, Cologne, Germany.
20
Département 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
Departement 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
Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation, Pavia, Italy.
23
Unidade de Doenças Auto-imunes/Medicina 7.2, Hospital de Curry Cabral, Centro Hospitalar de Lisboa Central, Lisbon, Portugal.
24
Service 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
Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, IRCCS Polyclinic Hospital San Martino, University of Genoa, Genoa, Italy.
26
Department of Rheumatology, Department of Internal Medicine, Ghent University Hospital, Ghent University, Ghent, Belgium.
27
University of Pisa, Pisa, Italy.
28
Department of Rheumatology and Clinical Immunology, Kerckhoff Klinik, Justus-Liebig University of Giessen, Bad Nauheim, Germany.
29
Department of Rheumatology, Universitätsklinikum Düsseldorf, Düsseldorf, Germany.
30
Liga Portuguesa Contra as Doenças Reumáticas, Núcleo Síndrome de Sjögren, Lisbon, Portugal.

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.

Supplemental Content

Full text links

Icon for BMJ Publishing Group Icon for PubMed Central
Loading ...
Support Center