PT  - JOURNAL ARTICLE
AU  - Alain Meyer
AU  - Carlo Alberto Scirè
AU  - Rosaria Talarico
AU  - Tobias Alexander
AU  - Zahir Amoura
AU  - Tadej Avcin
AU  - Simone Barsotti
AU  - Lorenzo Beretta
AU  - Jelena Blagojevic
AU  - Gerd Burmester
AU  - Ilaria Cavazzana
AU  - Patrick Cherrin
AU  - Laura Damian
AU  - Andrea Doria
AU  - João Eurico Fonseca
AU  - Federica Furini
AU  - Ilaria Galetti
AU  - Frederic Houssiau
AU  - Thomas Krieg
AU  - Maddalena Larosa
AU  - David Launay
AU  - Raquel Campanilho-Marques
AU  - Thierry Martin
AU  - Marco Matucci-Cerinic
AU  - Pia Moinzadeh
AU  - Carlomaurizio Montecucco
AU  - Maria Francisca Moraes-Fontes
AU  - Luc Mouthon
AU  - Rossella Neri
AU  - Sabrina Paolino
AU  - Yves Piette
AU  - Simona Rednic
AU  - Farah Tamirou
AU  - Angela Tincani
AU  - Natasa Toplak
AU  - Stefano Bombardieri
AU  - Eric Hachulla
AU  - Ulf Mueller-Ladner
AU  - Matthias Schneider
AU  - Vanessa Smith
AU  - Ana Vieira
AU  - Maurizio Cutolo
AU  - Marta Mosca
AU  - Lorenzo Cavagna
TI  - Idiopathic inflammatory myopathies: state of the art on clinical practice guidelines
AID  - 10.1136/rmdopen-2018-000784
DP  - 2019 Feb 01
TA  - RMD Open
PG  - e000784
VI  - 4
IP  - Suppl 1
4099  - http://rmdopen.bmj.com/content/4/Suppl_1/e000784.short
4100  - http://rmdopen.bmj.com/content/4/Suppl_1/e000784.full
SO  - RMD Open2019 Feb 01; 4
AB  - 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.