. 2018 Oct 18;4(Suppl 1):e000788.

doi: 10.1136/rmdopen-2018-000788. eCollection 2018.

Relapsing polychondritis: state of the art on clinical practice guidelines

Simona Rednic¹, Laura Damian¹, Rosaria Talarico², Carlo Alberto Scirè³, Alexander Tobias⁴, Nathalie Costedoat-Chalumeau^{5

6

7}, David Launay⁸, Alexis Mathian⁹, Lisa Mattews¹⁰, Cristina Ponte¹¹, Paola Toniati¹², Stefano Bombardieri¹³, Charissa Frank¹⁴, Matthias Schneider¹⁵, Vanessa Smith^{16

17}, Maurizio Cutolo¹⁸, Marta Mosca^{2

19}, Laurent Arnaud²⁰

Affiliations

¹ Department of Rheumatology, Emergency County Teaching Hospital, Cluj-Napoca, Romania.
² Rheumatology Unit, AOU Pisana, Pisa, Italy.
³ Department of Medical Sciences, Section of Rheumatology, University of Ferrara, Ferrara, Italy.
⁴ Department of Rheumatology and Clinical Immunology, Charité University Hospital Berlin, Berlin, Germany.
⁵ Assistance Publique-Hôpitaux de Paris (AP-HP), Cochin Hospital, Internal Medicine Department, Referral Center for Rare Autoimmune and Systemic Diseases, Paris, France.
⁶ Paris Descartes-Sorbonne Paris Cité University, Paris, France.
⁷ INSERM U 1153, Center for Epidemiology and Statistics Sorbonne Paris Cité (CRESS), Paris, France.
⁸ Département de Médecine Interne et Immunologie Clinique, Centre de Référence des Maladies Systémiques et Auto-Immunes Rares du Nord-Ouest (CERAINO), LIRIC, INSERM, Univ. Lille, CHU Lille, Lille, France.
⁹ Department of Internal Medicine, Hospital Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris, Paris, France.
¹⁰ Relapsing Polychondritis Awareness and Support, Worcester, UK.
¹¹ Department of Rheumatology, Hospital de Santa Maria, Centro Hospitalar Lisboa Norte, Lisbon Academic Medical Centre, Lisbon, Portugal.
¹² Rheumatology and Clinical Immunology Unit, Civil Hospital, Brescia, Italy.
¹³ University of Pisa, Pisa, Italy.
¹⁴ Bindweefsel.be, Koersel, Belgium.
¹⁵ Department of Rheumatology, Universitätsklinikum Düsseldorf, Düsseldorf, Germany.
¹⁶ Department of Rheumatology, Ghent University Hospital, Ghent, Belgium.
¹⁷ Department of Internal Medicine, Ghent University, Ghent, Belgium.
¹⁸ Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, IRCCS Polyclinic Hospital San Martino, University of Genoa, Genoa, Italy.
¹⁹ Rheumatology Unit, University of Pisa, Pisa, Italy.
²⁰ Service de rhumatologie, Hôpitaux Universitaires de Strasbourg, Centre National de Référence des Maladies Systémiques et Auto-immunes Rares (RESO), INSERM-UMRS 1109, F-67000, Strasbourg, France.

PMID: 30402273
PMCID: PMC6203097
DOI: 10.1136/rmdopen-2018-000788

Relapsing polychondritis: state of the art on clinical practice guidelines

Simona Rednic et al. RMD Open. 2018.

. 2018 Oct 18;4(Suppl 1):e000788.

doi: 10.1136/rmdopen-2018-000788. eCollection 2018.

Authors

Affiliations

¹ Department of Rheumatology, Emergency County Teaching Hospital, Cluj-Napoca, Romania.
² Rheumatology Unit, AOU Pisana, Pisa, Italy.
³ Department of Medical Sciences, Section of Rheumatology, University of Ferrara, Ferrara, Italy.
⁴ Department of Rheumatology and Clinical Immunology, Charité University Hospital Berlin, Berlin, Germany.
⁵ Assistance Publique-Hôpitaux de Paris (AP-HP), Cochin Hospital, Internal Medicine Department, Referral Center for Rare Autoimmune and Systemic Diseases, Paris, France.
⁶ Paris Descartes-Sorbonne Paris Cité University, Paris, France.
⁷ INSERM U 1153, Center for Epidemiology and Statistics Sorbonne Paris Cité (CRESS), Paris, France.
⁸ Département de Médecine Interne et Immunologie Clinique, Centre de Référence des Maladies Systémiques et Auto-Immunes Rares du Nord-Ouest (CERAINO), LIRIC, INSERM, Univ. Lille, CHU Lille, Lille, France.
⁹ Department of Internal Medicine, Hospital Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris, Paris, France.
¹⁰ Relapsing Polychondritis Awareness and Support, Worcester, UK.
¹¹ Department of Rheumatology, Hospital de Santa Maria, Centro Hospitalar Lisboa Norte, Lisbon Academic Medical Centre, Lisbon, Portugal.
¹² Rheumatology and Clinical Immunology Unit, Civil Hospital, Brescia, Italy.
¹³ University of Pisa, Pisa, Italy.
¹⁴ Bindweefsel.be, Koersel, Belgium.
¹⁵ Department of Rheumatology, Universitätsklinikum Düsseldorf, Düsseldorf, Germany.
¹⁶ Department of Rheumatology, Ghent University Hospital, Ghent, Belgium.
¹⁷ Department of Internal Medicine, Ghent University, Ghent, Belgium.
¹⁸ Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, IRCCS Polyclinic Hospital San Martino, University of Genoa, Genoa, Italy.
¹⁹ Rheumatology Unit, University of Pisa, Pisa, Italy.
²⁰ Service de rhumatologie, Hôpitaux Universitaires de Strasbourg, Centre National de Référence des Maladies Systémiques et Auto-immunes Rares (RESO), INSERM-UMRS 1109, F-67000, Strasbourg, France.

PMID: 30402273
PMCID: PMC6203097
DOI: 10.1136/rmdopen-2018-000788

Abstract

Due to the rarity of relapsing polychondritis (RP), many unmet needs remain in the management of RP. Here, we present a systematic review of clinical practice guidelines (CPGs) published for RP, as well as a list of the most striking unmet needs for this rare disease. We carried out a systematic search in PubMed and Embase based on controlled terms (medical subject headings and Emtree) and keywords of the disease and publication type (CPGs). The systematic literature review identified 20 citations, among which no CPGs could be identified. We identified 11 main areas with unmet needs in the field of RP: the diagnosis strategy for RP; the therapeutic management of RP; the management of pregnancy in RP; the management of the disease in specific age groups (for instance in paediatric-onset RP); the evaluation of adherence to treatment; the follow-up of patients with RP, including the frequency of screening for the potential complications and the optimal imaging tools for each involved region; perioperative and anaesthetic management (due to tracheal involvement); risk of neoplasms in RP, including haematological malignancies; the prevention and management of infections; tools for assessment of disease activity and damage; and patient-reported outcomes and quality of life indicators. Patients and physicians should work together within the frame of the ReCONNET network to derive valuable evidence for obtaining literature-informed CPGs.

Keywords: ReCONNET; clinical practice guidelines; european references networks; relapsing polychondritis; unmet needs.

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Conflict of interest statement

Competing interests: None declared.

Figures

**Figure 1**
Flow chart showing the study selection process.

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References

1. Lahmer T, Treiber M, von Werder A, et al. . Relapsing polychondritis: an autoimmune disease with many faces. Autoimmun Rev 2010;9:540–6. 10.1016/j.autrev.2010.02.016 - DOI - PubMed
1. Jaksch-Wartenhorst R. Polychondropathia. Wien Arch F Inn Med 1923;6:93–100.
1. Pearson CM, Kline HM, Newcomer VD. Relapsing polychondritis. N Engl J Med 1960;263:51–8. 10.1056/NEJM196007142630201 - DOI - PubMed
1. Michet CJ, McKenna CH, Luthra HS, et al. . Relapsing polychondritis. Survival and predictive role of early disease manifestations. Ann Intern Med 1986;104:74–8. - PubMed
1. Arnaud L, Mathian A, Haroche J, et al. . Pathogenesis of relapsing polychondritis: a 2013 update. Autoimmun Rev 2014;13:90–5. 10.1016/j.autrev.2013.07.005 - DOI - PubMed

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Relapsing polychondritis: state of the art on clinical practice guidelines

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Relapsing polychondritis: state of the art on clinical practice guidelines

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