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RMD Open. 2018 Oct 18;4(Suppl 1):e000783. doi: 10.1136/rmdopen-2018-000783. eCollection 2018.

Mixed connective tissue disease: state of the art on clinical practice guidelines.

Author information

1
Service de Médecine Interne, Hôpital Cochin, Centre de Référence Maladies systémiques Autoimmunes Rares d'Ile de France, Assistance Publique-Hôpitaux de Paris (AP-HP), Université Paris Descartes, Paris, France.
2
Section of Rheumatology, Department of Medical Sciences, University of Ferrara, Ferrara, Italy.
3
Rheumatology Unit, AOU Pisana, Pisa, Italy.
4
Department of Rheumatology and Clinical Immunology, Charité University Hospital Berlin, Berlin, Germany.
5
Department of Internal Medicine, Hospital Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris, Paris, France.
6
Department of Allergology Rheumatology and Clinical Immunology, University Children's Hospital, University Medical Centre Ljubljana, Ljubljana, Slovenia.
7
Referral Center for Systemic Autoimmune Diseases, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico di Milano, Milan, Italy.
8
Rheumatology Unit Department of Medicine, AO Padova and University of Padua, Padua, Italy.
9
Service de rhumatologie Hôpitaux Universitaires de Strasbourg, Centre National de Référence des Maladies Systémiques et Auto-immunes Rares Grand-Est Sud-Ouest, Strasbourg, France.
10
Liga Portuguesa Contra as Doenças Reumáticas, Lisbon, Portugal.
11
Département de Médecine Interne et Immunologie Clinique, Centre de Référence des Maladies Systémiques et Auto-Immunes Rares du Nord-Ouest, Lille, France.
12
Department of Dermatology, Universitätsklinikum Köln, Cologne, Germany.
13
Division of Rheumatology and Scleroderma Unit, Department of Clinical and Experimental Medicine AOU Careggi, University of Florence, Florence, Italy.
14
Department of Rheumatology and Clinical Immunology, Kerckhoff Klinik, Justus-Liebig University of Giessen, Bad Nauheim, Germany.
15
Department of Rheumatology, Emergency County Teaching Hospital, Cluj-Napoca, Romania.
16
Centro Hospitalar de Lisboa Central, Lisbon, Portugal.
17
Amsterdam Rheumatology & immunology Center (ARC), Academic Medical Center, Amsterdam, The Netherlands.
18
Clinical Immunology & Rheumatology, Amsterdam Rheumatology & Immunology Center, Academic Medical Center/University of Amsterdam, Amsterdam, The Netherlands.
19
Liga Portuguesa Contra as Doenças Reumáticas, Núcleo Síndrome de Sjögren, Lisbon, Portugal.
20
University of di Pisa, Pisa, Italy.
21
Rheumatology Department Hospital de Santa Maria, Centro Hospitalar Lisboa Norte, Lisbon Academic Medical Centre, Libson, Portugal.
22
FESCA, Federation of European Scleroderma Associations, Milan, Italy.
23
Department of Rheumatology, Universitätsklinikum Düsseldorf, Düsseldorf, Germany.
24
Department of Rheumatology, Ghent University Hospital, Ghent, Belgium.
25
Department of Internal Medicine, Ghent University, Ghent, Belgium.
26
Research Laboratory and Academic Division of Clinical Rheumatology Department of Internal Medicine, IRCCS Polyclinic Hospital San Martino, University of Genoa, Genoa, Italy.

Abstract

Mixed connective tissue disease (MCTD) is a complex overlap disease with features of different autoimmune connective tissue diseases (CTDs) namely systemic sclerosis, poly/dermatomyositis and systemic lupus erythematous in patients with antibodies targeting the U1 small nuclear ribonucleoprotein particle. 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 (CPGs) or recommendations. Since no specific CPGs on MCTD were found, other CPGs developed for other CTDs were taken into consideration in order to discuss what can be applied to MCTD even if designed for other diseases. Three major objectives were proposed for the future development of CPGs: MCTD diagnosis (diagnostic criteria), MCTD initial and follow-up evaluations, MCTD treatment. Early diagnosis, epidemiological data, assessment of burden of disease and QOL aspects are among the unmet needs identified by patients.

KEYWORDS:

ERN reconnet; clinical practice guidelines; european reference networks; mixed connective tissue disease; unmet needs

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