RT Journal Article
SR Electronic
T1 Relapsing polychondritis: state of the art on clinical practice guidelines
JF RMD Open
JO RMD Open
FD EULAR
SP e000788
DO 10.1136/rmdopen-2018-000788
VO 4
IS Suppl 1
A1 Simona Rednic
A1 Laura Damian
A1 Rosaria Talarico
A1 Carlo Alberto Scirè
A1 Alexander Tobias
A1 Nathalie Costedoat-Chalumeau
A1 David Launay
A1 Alexis Mathian
A1 Lisa Matthews
A1 Cristina Ponte
A1 Paola Toniati
A1 Stefano Bombardieri
A1 Charissa Frank
A1 Matthias Schneider
A1 Vanessa Smith
A1 Maurizio Cutolo
A1 Marta Mosca
A1 Laurent Arnaud
YR 2018
UL http://rmdopen.bmj.com/content/4/Suppl_1/e000788.abstract
AB 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.