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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 Connective tissue diseases RMD Open: first published as 10.1136/rmdopen-2018-000788 on 18 October 2018. Downloaded from REVIEW Relapsing polychondritis: state of the art on clinical practice guidelines Simona Rednic,1 Laura Damian,1 Rosaria Talarico,2 Carlo Alberto Scirè,3 Alexander Tobias,4 Nathalie Costedoat-Chalumeau,5,6,7 David Launay,8 Alexis Mathian,9 Lisa Matthews,10 Cristina Ponte,11 Paola Toniati,12 Stefano Bombardieri,13 Charissa Frank,14 Matthias Schneider,15 Vanessa Smith,16,17 Maurizio Cutolo,18 Marta Mosca,2,19 Laurent Arnaud20 To cite: Rednic S, Damian L, ABSTRACT Key messages Talarico R, et al. Relapsing Due to the rarity of relapsing polychondritis (RP), many polychondritis: state of unmet needs remain in the management of RP. Here, we What is already known about this subject? the art on clinical practice present a systematic review of clinical practice guidelines guidelines. RMD Open Due to the rarity of the disease and the paucity of (CPGs) published for RP, as well as a list of the most ► 2018;4:e000788. doi:10.1136/ available evidence, many unmet needs remain in the striking unmet needs for this rare disease. We carried out rmdopen-2018-000788 field of relapsing polychondritis (RP). a systematic search in PubMed and Embase based on controlled terms (medical subject headings and Emtree) What does this study add? ► Prepublication history for and keywords of the disease and publication type (CPGs). ► We performed a systematic review of clinical prac- this paper is available online. The systematic literature review identified 20 citations, To view these files, please visit tice guidelines published for RP, and we identified among which no CPGs could be identified. We identified the journal online (http:// dx. doi. the most striking unmet needs in this rare disease. org/ 10. 1136/ rmdopen- 2018- 11 main areas with unmet needs in the field of RP: the 000788). diagnosis strategy for RP; the therapeutic management of How might this impact on clinical practice? RP; the management of pregnancy in RP; the management ► Due to the extreme rarity of the disease, patients and SR and LD are co-authors. of the disease in specific age groups (for instance in physicians should work together within the frame paediatric-onset RP); the evaluation of adherence to of the ReCONNET network to derive valuable evi- Received 6 August 2018 treatment; the follow-up of patients with RP, including the dence for informing literature-based clinical practice Revised 31 August 2018 frequency of screening for the potential complications guidelines for RP. Accepted 7 September 2018 and the optimal imaging tools for each involved region; http://rmdopen.bmj.com/ perioperative and anaesthetic management (due to tracheal involvement); risk of neoplasms in RP, including three of the auricular, nasal or laryngotra- haematological malignancies; the prevention and cheal cartilages, or proven inflammation in management of infections; tools for assessment of disease activity and damage; and patient-reported outcomes and one cartilage plus two other signs, including quality of life indicators. Patients and physicians should ocular inflammation, vestibular dysfunc- work together within the frame of the ReCONNET network tion, seronegative inflammatory arthritis or to derive valuable evidence for obtaining literature- hearing loss. RP is a chronic disease with a informed CPGs. flaring-remitting course. The exact cause of on September 24, 2021 by guest. Protected copyright. RP is still unknown, but the disease is mostly regarded as an immune-mediated disease,5 as there is a well-documented overlap of INTRODUCTION RP with other rheumatic and autoimmune Relapsing polychondritis (RP) is a systemic diseases.4 6 Moreover, RP is strongly associ- inflammatory disease primarily affecting ated with the Human Leucocyte Antigen © Author(s) (or their the cartilaginous structures of the ears, (HLA) allele DR4, and various immune employer(s)) 2018. Re-use nose and tracheobronchial tree, but also responses directed against cartilage compo- permitted under CC BY-NC. No the joints, the inner ear, the eyes and the nents have been demonstrated in patients commercial re-use. See rights 1 5 and permissions. Published cardiovascular system. The first case of RP with RP. Since RP is a very rare disease, with by BMJ. was described in 1923 by Jaksch-Warten- a prevalence estimated to be as low as a few 2 7 8 For numbered affiliations see horst, but little attention has been given cases per million, it remains an under-re- end of article. to the entity until the 1960s, when Pearson searched area. Scores for assessing both et al3 introduced the name ‘relapsing poly- disease activity and damage in RP have been Correspondence to Professor Laurent Arnaud; chondritis’. The classification criteria for developed by our group, with the help of a 4 9 laurent. arnaud@ chru- RP by Michet et al require the presence panel of international experts. Importantly, strasbourg. fr of proven inflammation in at least two of due to the rarity of the disease, the lack of Rednic S, et al. RMD Open 2018;4:e000788. doi:10.1136/rmdopen-2018-000788 1 RMD Open RMD Open: first published as 10.1136/rmdopen-2018-000788 on 18 October 2018. Downloaded from Figure 1 Flow chart showing the study selection process. adequate networks of care for RP in most countries and Evaluation II (AGREE II) tool checklist not for formal the paucity of drug efficacy data, many unmet needs appraisal but only to inform discussion. A discussion remain in the field of RP. Here we present a systematic group was set for the evaluation of the existing CPGs review of clinical practice guidelines (CPGs) published and to identify the unmet needs. for RP, as well as a list of the most striking unmet needs Here is the search strategy: Medline (PubMed): (“poly- in this rare disease. The review has been carried out chondritis, relapsing” [MeSH Terms] OR (“polychondritis” under the framework of the European Reference [All Fields] AND “relapsing” [All Fields]) OR “relapsing Network on Rare and Complex Connective Tissue and polychondritis” [All Fields] OR (“relapsing” [All Fields] AND Musculoskeletal Diseases (ERN ReCONNET), gath- “polychondritis” [All Fields]) AND (“Practice Guideline” ering the experience of several European centres with [Publication Type] OR “Practice Guidelines As Topic” [MeSH http://rmdopen.bmj.com/ experience in the diagnosis and follow-up of RP and Terms] OR Practice Guideline [Publication Type] OR “Prac- involving patient representatives. tice Guideline” [Text Word] OR “Practice Guidelines” [Text Word] OR “Guideline” [Publication Type] OR “Guidelines METHODS As Topic” [MeSH Terms] OR Guideline [Publication Type] We carried out a systematic search in PubMed and OR “Guideline” [Text Word] OR “Guidelines” [Text Word] Embase based on controlled terms (medical subject OR “Consensus Development Conference” [Publication Type] headings (MeSH) and Emtree) and keywords of the OR “Consensus Development Conferences As Topic” [MeSH disease and publication type (CPGs). We reviewed all Terms] OR “Consensus” [MeSH Terms] OR “Consensus” on September 24, 2021 by guest. Protected copyright. the published articles in order to identify existing CPGs [Text Word] OR “Recommendation” [Text Word] OR “Recom- on diagnosis, monitoring and treatment, according to mendations” [Text Word] OR “Best Practice” [Text Word] OR the definition of the Institute of Medicine 2011 (CPGs “Best Practices” [Text Word]). Embase: (‘relapsing polychon- are statements that include recommendations intended dritis’/exp OR ‘atrophic polychondritis’ OR ‘chronic atrophic to optimise patient care that are informed by a system- polychondritis’ OR ‘chronic atropic polychondritis’ OR ‘chronic atic review of evidence and an assessment of the bene- polychondritis, atrophic’ OR ‘polychondritis, relapsing’ OR fits and harms of alternative care options). The disease ‘relapsing polychondritis’) AND (‘practice guideline’/exp OR coordinator (DC) of the ERN ReCONNET for RP has ‘practice guideline’ OR ‘practice guidelines’/exp OR ‘practice assigned the work on CPGs to the healthcare providers guidelines’ OR ‘clinical practice guideline’/exp OR ‘clinical involved. Moreover, in order to implement the list practice guideline’ OR ‘clinical practice guidelines’/exp OR of guidelines provided by the Medline and Embase ‘clinical practice guidelines’ OR ‘clinical practice guidelines search, the group also performed a hand search. A as topic’/exp OR ‘clinical practice guidelines as topic’ OR first screening among papers included in the final list ‘guideline’/exp OR ‘guideline’ OR ‘guidelines’/exp OR ‘guide- (systematic search + hand search) based on title and lines’ OR ‘guidelines as topic’/exp OR ‘guidelines as topic’ OR abstract selected evidence-based medicine guidelines. ‘consensus development’/exp OR ‘consensus development’ OR A general assessment of the CPGs has been performed ‘consensus development conference’/exp OR ‘consensus devel- following the Appraisal of Guidelines for Research & opment conference’ OR ‘consensus development conferences’/ 2 Rednic S, et al. RMD Open 2018;4:e000788. doi:10.1136/rmdopen-2018-000788 Connective tissue diseases RMD Open: first published as 10.1136/rmdopen-2018-000788 on 18 October 2018. Downloaded from granulomatosis with polyangiitis (GPA), T-cell lymphoma, Box 1 Unmeet needs in the field of relapsing sarcoidosis and so on have to be differentiated from polychondritis (RP) RP. Moreover, another immune-mediated disease, such Major unmet needs in the field
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