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Joint Bone Spine
Volume 86, n° 3
pages 363-368 (mai 2019)
Doi : 10.1016/j.jbspin.2018.11.001
accepted : 6 November 2018
Original Articles

The relapsing polychondritis damage index (RPDAM): Development of a disease-specific damage score for relapsing polychondritis

Philippe Mertz a, b, Alexandre Belot c, Ricard Cervera d, Tyng Yu Chuah e, Lorenzo Dagna f, Laura Damian g, Debashish Danda h, David D'cruz i, Gerard Espinosa d, Camille Frances j, David Jayne k, Kong Kok Ooi l, m, Eugene J. Kucharz n, Robert Lebovics o, Isabelle Marie p, Guillaume Moulis q, r, Stanford Peng s, Aman Sharma t, Noboru Suzuki u, Toshio Tanaka v, Ronald Van Vollenhoven w, Jean Sibilia a, b, Jacques Eric Gottenberg a, b, François Chasset j, Laurent Arnaud a, b,
a Service de rhumatologie, centre de référence des maladies autoimmunes systemiques rares Est Sud-Ouest (RESO), hôpital de Hautepierre, 1, avenue Molière BP 83049, 67098 Strasbourg cedex, France 
b Inserm UMR-S1109, 67098 Strasbourg cedex, France 
c Department of paediatric nephrology, rheumatology, dermatology, hôpital Femme-Mère Enfant, Bron, France 
d Department of autoimmune diseases, institut clinic de medicina i dermatología, hospital clínic, Barcelona, Spain 
e Department of rheumatology and immunology, Singapore general hospital, Singapore 
f Unit of immunology, rheumatology, allergy and rare diseases (UnIRAR), IRCCS San Raffaele scientific institute, Vita-Salute San Raffaele university, 20132 Milan, Italy 
g Rheumatology department, Spitalul clunic Judetean de Urgenta Cluj. Cluj-Napoca, Romania 
h Department of clinical immunology and rheumatology, Christian medical college, Vellore, India 
i Louise Coote Lupus Unit, Guy's Hospital, London, UK 
j Dermatology department, hôpital Tenon, Paris, France 
k Department of medicine, university of Cambridge, Cambridge, UK 
l Division of rheumatology, national university hospital, Singapore city, Singapore 
m Yong Loo Lin school of medicine, national university, Singapore city, Singapore 
n Department of internal medicine and rheumatology, medical university of Silesia, Katowice, Poland 
o Department of otolaryngology, from Mount Sinai St. Luke's and Mount Sinai Roosevelt affiliated with the Icahn school of medicine at Mount Sinai, New York, NY, United States 
p Department of internal medicine, CHU de Rouen-Bois Guillaume, Rouen cedex, France 
q Department of internal medicine, CHU de Toulouse, Toulouse, France 
r UMR 1027 Inserm-university of Toulouse, CIC 1436, CHU de Toulouse, Toulouse, France 
s Swedish Medical Center, Seattle, Washington, USA 
t Clinical immunology and rheumatology services, department of internal medicine, postgraduate institute of medical education and research (PGIMER), Chandigarh, 160012, India 
u Institute of medical science and department of immunology and medicine, St. Marianna University school of medicine, Kawasaki, 216-8511, Japan 
v Department of clinical application of biologics, Osaka university graduate school of medicine, Osaka University, Osaka 565-0871, Japan 
w Department of clinical immunology and rheumatology, academic medical center, Amsterdam, The Netherlands 

Corresponding author at: Service de rhumatologie, centre national de reference des maladies autoimmunes et systemiques rares, hôpital de Hautepierre, 1, avenue Molière BP 83049, 67098 Strasbourg cedex, France.Service de rhumatologie, centre national de reference des maladies autoimmunes et systemiques rares, hôpital de Hautepierre1, avenue Molière BP 83049Strasbourg cedex67098France

Relapsing polychondritis is a rare, multi-systemic and inflammatory condition of unknown origin. We currently lack a core set of measures to assess and follow damage in patients suffering from this condition. Our primary aim was to derive a disease-specific damage measuring tool for relapsing polychondritis, the Relapsing Polychondritis Damage Index (RPDAM).


We performed an international 4-round multicenter Delphi study during which experts were asked to rate the relevance of potential damage items for relapsing polychondritis (141 items were obtained from a literature review and 12 from expert suggestion), using a Likert Scale. The selection of items for each subsequent round was based on the median rating of each item.


Twenty-four experts from 11 nationalities participated in round 1 and 22 in rounds 2, 3 and 4. From the initial 153 potential damage items, 44 items were selected during round 1, 30 items during round 2 and 16 during round 3. During round 4, we refined the index to a total of 17 items referring to ear nose and throat, eye, respiratory, cardiovascular and hematological systems as well as to treatment-related specific damage items.


We have developed by international consensus a scoring system to assess damage in patients with relapsing polychondritis. Following its validation, the RPDAM may contribute to improve the care of patients suffering from this rare condition as well as to standardize data collection for future clinical trials.

The full text of this article is available in PDF format.

Keywords : Autoinflammatory diseases, Damage Index, Relapsing polychondritis

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