Rheumatology International

, Volume 38, Issue 11, pp 2095–2101 | Cite as

Observational study and brief analysis of diagnostic criteria in relapsing polychondritis

  • Thomas RoseEmail author
  • Udo Schneider
  • Martina Bertolo
  • Jens Klotsche
  • Vincent Casteleyn
  • Robert Biesen
  • Gerd R. Burmester
  • Falk Hiepe
Observational Research


Observational study of patients with relapsing polychondritis (RPC) and brief evaluation of widely used diagnostic criteria. A retrospective analysis of 18 patients with RPC treated in the past 15 years at the Charté—Universitätsmedizin Berlin was performed. Three different diagnostic criteria were applied to our cohort. Sensitivities of diagnostic criteria of McAdam et al., Damiani and Levine and Michet et al. were calculated as well as the 5- and 10-year survival. Analysis of diagnostic criteria revealed a sensitivity of 88.9% using Damiani and Levine criteria, 66.7% for Michet et al. and 50% for McAdam et al., respectively. Modifying the criteria of Michet et al. increases the sensitivity to 88.9%. The 5- and 10-year survival were 100 and 90.9%, respectively. Current diagnostic criteria in RPC should be reappraised covering the diversity of clinical findings with the aim to improve clinical care and research in RPC.


Relapsing polychondritis Survival Diagnosis Criteria Inflammation 



The authors thank Anna R. Lisney for proof-reading of the manuscript.

Compliance with ethical standards

Conflict of interest

All authors have no financial or competing interests to declare. We received no financial support or other benefits from commercial sources for the reported work on the manuscript.


  1. 1.
    Mathian A, Miyara M, Cohen-Aubart F et al (2016) Relapsing polychondritis: a 2016 update on clinical features, diagnostic tools, treatment and biological drug use. Best Pract Res Clin Rheumatol 30:316–333. CrossRefPubMedGoogle Scholar
  2. 2.
    Lekpa FK, Chevalier X (2018) Refractory relapsing polychondritis: challenges and solutions. In: Open Access Rheumatol. Res. Rev. Accessed 20 Mar 2018
  3. 3.
    Arnaud L, Mathian A, Haroche J et al (2014) Pathogenesis of relapsing polychondritis: a 2013 update. Autoimmun Rev 13:90–95. CrossRefPubMedGoogle Scholar
  4. 4.
    FrancèS C, El Rassi R, Laporte JL et al (2001) Dermatologic manifestations of relapsing polychondritis: a study of 200 cases at a single center. Medicine (Baltimore) 80:173–179. CrossRefGoogle Scholar
  5. 5.
    Michet CJ (1986) Relapsing polychondritis: survival and predictive role of early disease manifestations. Ann Intern Med 104:74. CrossRefPubMedGoogle Scholar
  6. 6.
    McAdam LP, O’Hanlan MA, Bluestone R, Pearson CM (1976) Relapsing polychondritis: prospective study of 23 patients and a review of the literature. Medicine (Baltimore) 55:193–215CrossRefGoogle Scholar
  7. 7.
    Buckley LM, Ades PA Progressive aortic valve inflammation occurring despite apparent remission of relapsing polychondritis. Arthritis Rheum 35:812–814. CrossRefGoogle Scholar
  8. 8.
    Cipriano PR, Alonso DR, Baltaxe HA et al (1976) Multiple aortic aneurysms in relapsing polychondritis. Am J Cardiol 37:1097–1102CrossRefGoogle Scholar
  9. 9.
    Dion Jérémie Costedoat-Chalumeau, Nathalie S, Damien et al (2016) Relapsing polychondritis can be characterized by three different clinical phenotypes: analysis of a recent series of 142 patients. Arthritis Rheumatol 68:2992–3001. CrossRefPubMedGoogle Scholar
  10. 10.
    Moulis G, Pugnet G, Costedoat-Chalumeau N et al (2018) Efficacy and safety of biologics in relapsing polychondritis: a French national multicentre study. Ann Rheum Dis. CrossRefPubMedGoogle Scholar
  11. 11.
    Kemta Lekpa F, Kraus VB, Chevalier X (2012) Biologics in relapsing polychondritis: a literature review. Semin Arthritis Rheum 41:712–719. CrossRefPubMedGoogle Scholar
  12. 12.
    Cantarini L, Vitale A, Brizi MG et al (2014) Diagnosis and classification of relapsing polychondritis. J Autoimmun 48–49:53–59. CrossRefPubMedGoogle Scholar
  13. 13.
    Damiani JM, Levine HL (1979) Relapsing polychondritis–report of ten cases. Laryngoscope 89:929–946CrossRefGoogle Scholar
  14. 14.
    Trentham DE (1998) Relapsing polychondritis. Ann Intern Med 129:114. CrossRefPubMedGoogle Scholar
  15. 15.
    Mathew SD, Battafarano DF, Morris MJ (2012) Relapsing polychondritis in the department of defense population and review of the literature. Semin Arthritis Rheum 42:70–83. CrossRefPubMedGoogle Scholar
  16. 16.
    Zeuner M, Straub RH, Rauh G et al (1997) Relapsing polychondritis: clinical and immunogenetic analysis of 62 patients. J Rheumatol 24:96–101PubMedGoogle Scholar
  17. 17.
    PIETTE J, EL-RASSI R, AMOURA Z (1999) Antinuclear antibodies in relapsing polychondritis. Ann Rheum Dis 58:656–657CrossRefGoogle Scholar

Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2018

Authors and Affiliations

  • Thomas Rose
    • 1
    Email author
  • Udo Schneider
    • 1
  • Martina Bertolo
    • 2
  • Jens Klotsche
    • 3
  • Vincent Casteleyn
    • 1
  • Robert Biesen
    • 1
  • Gerd R. Burmester
    • 1
  • Falk Hiepe
    • 1
  1. 1.Department of Rheumatology and Clinical ImmunologyCharité Universitätsmedizin BerlinBerlinGermany
  2. 2.Department of NephrologyCharité Universitätsmedizin BerlinBerlinGermany
  3. 3.German Rheumatism Research CenterBerlin-Leibniz InstituteBerlinGermany

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