Relapsing Polychondritis

  • Harvinder S. Luthra


Relapsing polychondritis is a recurring inflammatory disorder of unknown etiology causing inflammatory reactions in the cartilaginous structures of the nose, ears, trachea, and the joints. It is considered an auto-immune disorder. It was first described by Jaksch-Wartenhorst (1), in a patient with systemic illness characterized by external ear swelling, collapse of the nasal bridge, fever, and arthritis. Pearson and colleagues (2) first coined the term relapsing polychondritis (RP), and described, in detail, the clinical features of several of their own patients along with those in the literature. Since then, this disorder has been described worldwide and occurs in all age groups, although it peaks in the fifth decade. Over 30% of patients have an associated disorder, usually autoimmune or hematologic. These include systemic vasculitis syndromes, systemic lupus erythematosus (SLE), Sjögren’s syndrome, overlap connective tissue disorders, rheumatoid arthritis (RA), spondyloarthropathies, dysmyelopoietic syndromes, Hodgkin’s disease, diabetes mellitis, and psoriasis vulgaris (3). It is a rare disease occuring in 3.5 person’s per million (Michet, personal communication). The 5-year survival is decreased to approximately 74%. The male:female ratio is equal (4).


Aortic Valve Disease Nasal Bridge Relapse Polychondritis Large Vessel Disease Nasal Cartilage 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


  1. 1.
    Jaksch-Wartenhorst R. Polychondropathia. Wien Arch F Inn Med 1923;6:93–100.Google Scholar
  2. 2.
    Pearson CM, Kline HM, Newcomer VD. Relapsing polychondritis. N Engl J Med 1960;263:51–58.CrossRefPubMedGoogle Scholar
  3. 3.
    Kent PD, Michet CJ, Luthra HS. Relapsing polychondritis. Curr Opin Rheumatol 2004;16:56–61.CrossRefPubMedGoogle Scholar
  4. 4.
    Michet CJ Jr, McKenna CH, Luthra HS, O’Fallon WW. Relapsing polychondritis; survival and predictive role of early disease manifestations. Ann Int Med 1986;104:74–78.PubMedGoogle Scholar
  5. 5.
    Riccieri V, Spadaro A, Taccari E, Zoppini A. A case of relapsing polychondritis: pathogenetic considerations Clin Exp Rheumatol 1988;6:95–96.Google Scholar
  6. 6.
    Homma S, Matsumoto T, Abe H, Fukuda Y, Nagano M, Suzuki M. Relapsing polychondritis: pathological and immunological findings in an autopsy case. Acta Pathol Jpn 1984;34:1137–1146.PubMedGoogle Scholar
  7. 7.
    McKenna CH, Luthra HS, Jordan RE. Hypocomplementemic ear effusion in relapsing polychondritis. Mayo Clin Proc 1976;51:495–497.PubMedGoogle Scholar
  8. 8.
    Foidart J, Abe S, Martin GR, et al. Antibodies to type II collagen in relapsing polychondritis. N Engl J Med 1978;299:1203–1207.CrossRefPubMedGoogle Scholar
  9. 9.
    Herman JH, Dennis MV. Immunopathologic studies in relapsing polychondritis. J Clin Invest 1973;52:549–558.CrossRefPubMedGoogle Scholar
  10. 10.
    Taneja V, Griffiths M, Behrens M, Luthra HS, David CS. Auricular chondritis in NOD.DQ8.A beta o (A(g7-/-)) transgenic mice resembles human relapsing polychondritis. J Clin Invest 2003;112:1843–1850.PubMedGoogle Scholar
  11. 11.
    Isaak BL, Liesegang TJ, Michet CJ Jr. Ocular and systemic findings in relapsing polychondritis. Ophthalmology 1986;93:681–689.PubMedGoogle Scholar
  12. 12.
    Krell WS, Staats BA, Hyatt RE. Pulmonary function in relapsing polychondritis. Am Rev Respir Dis 1986;133:1120–1123.PubMedGoogle Scholar
  13. 13.
    Delrosso A, Petix NR, Pratesi M, et al. Cardiovascular involvement in relapsing polychondritis. Semin Arthritis Rheum 1997;26:840–844.CrossRefGoogle Scholar
  14. 14.
    O’Hanlon M, McAdam LP, Bluestone R, Pearson CM. The arthropathy of relapsing polychondritis. Arthritis Rheumatism 1976;19:191–194.CrossRefGoogle Scholar
  15. 15.
    Chang-Miller A, Okamura M, Torres VE, et al. Renal involvement in relapsing polychondritis. Medicine 1987;66:202–217.CrossRefPubMedGoogle Scholar
  16. 16.
    Myers B, Gould J, Dolan G. Relapsing polychondritis and myelodysplasia: a report of two cases and review of the literature. Clin Lab Haematol 2000;22:45–48.CrossRefPubMedGoogle Scholar
  17. 17.
    McAdam LP, O’Hanlon MA, Bluestone R, Pearson CM. Relapsing polychondritis: prospective study of 23 patients and a review of the literature. Medicine 1976;55:193–215.CrossRefPubMedGoogle Scholar
  18. 18.
    Booth A, Dieppe PA, Goddard PL, Watt I. The radiological manifestations of relapsing polychondritis. Clin Radiol 1989;40:147–149.CrossRefPubMedGoogle Scholar
  19. 19.
    Mendelson DS, Som PM, Crane R, Cohen BA, Spiera H. Relapsing polychondritis studied by computed tomography. Radiology 1985;157:489–490.PubMedGoogle Scholar
  20. 20.
    Davis SD, Berkmen YM, King T. Peripheral bronchial involvement in relapsing polychondritis: demonstration by thin-section CT. AJR Am J Roentgenol 1989;153:953–954.PubMedGoogle Scholar
  21. 21.
    Eng J, Sabanathan S. Airway complications of relapsing polychondritis. Ann Thorac Surg 1991;51:686–692.CrossRefPubMedGoogle Scholar
  22. 22.
    Buckley LM, Ades PA. Progressive aortic valve inflammation occuring despite apparent remission of relapsing polychondritis Arthritis Rheum 1992;35:812–814.CrossRefPubMedGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2008

Authors and Affiliations

  • Harvinder S. Luthra
    • 1
  1. 1.Department of RheumatologyMayo Clinic College of MedicineRochesterUSA

Personalised recommendations