Official reprint from UpToDate®
www.uptodate.com ©2016 UpToDate®

Clinical manifestations of relapsing polychondritis

Clement J Michet, MD
Section Editor
Peter H Schur, MD
Deputy Editor
Paul L Romain, MD


Relapsing polychondritis (RPC) is an immune-mediated condition associated with inflammation in cartilaginous structures and other tissues throughout the body, particularly the ears, nose, eyes, joints, and respiratory tract. Approximately one-third of RPC cases occur in association with another disease, usually some form of systemic vasculitis, a connective tissue disorder (systemic autoimmune rheumatologic disease), or a myelodysplastic syndrome (MDS).

The clinical features and course of RPC vary considerably from patient to patient. Subtle, early manifestations often remain unrecognized for prolonged periods. As a result, the diagnosis is frequently obtained only after the emergence of classic features such as auricular inflammation, saddle-nose deformity, or other features of cartilage destruction. No known clinical or laboratory measures predict the expression of specific disease manifestations or the overall disease course.

The clinical features of RPC are reviewed here. The pathogenesis, pathology, diagnosis, and treatment of RPC are discussed separately. (See "Etiology and pathogenesis of relapsing polychondritis" and "Pathology of relapsing polychondritis" and "Diagnostic evaluation of relapsing polychondritis" and "Treatment of relapsing polychondritis".)


Relapsing polychondritis (RPC) appears to occur among individuals of all races and age groups, and men and women are affected equally, but much about the epidemiology of RPC remains unknown because of the relatively small number of affected patients. RPC is most prevalent in Caucasians. The incidence in Rochester, Minnesota is estimated to be 3.5 per million population per year [1]. Onset is most likely between the ages of 40 and 60, although RPC can occur in childhood (figure 1) [2].

RPC is not a familial disease, although studies indicate that some genetic contribution to susceptibility is likely. One case report suggested the possibility of placental transmission [3]. However, no other cases of this occurrence are confirmed, and the reported association may be secondary to chance or a confluence of other circumstances. In general, cases suggesting placental transmission are exceptionally rare, even if the mother may be experiencing active multisystem disease [3].


Subscribers log in here

To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information or to purchase a personal subscription, click below on the option that best describes you:
Literature review current through: Sep 2016. | This topic last updated: Jul 22, 2016.
The content on the UpToDate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. The use of this website is governed by the UpToDate Terms of Use ©2016 UpToDate, Inc.
  1. Kent PD, Michet CJ Jr, Luthra HS. Relapsing polychondritis. Curr Opin Rheumatol 2004; 16:56.
  2. Knipp S, Bier H, Horneff G, et al. Relapsing polychondritis in childhood--case report and short review. Rheumatol Int 2000; 19:231.
  3. Gimovsky ML, Nishiyama M. Relapsing polychondritis in pregnancy: a case report and review. Am J Obstet Gynecol 1989; 161:332.
  4. Esdaile J, Hawkins D, Gold P, et al. Vascular involvement in relapsing polychondritis. Can Med Assoc J 1977; 116:1019.
  5. Michet CJ. Vasculitis and relapsing polychondritis. Rheum Dis Clin North Am 1990; 16:441.
  6. Selim AG, Fulford LG, Mohiaddin RH, Sheppard MN. Active aortitis in relapsing polychondritis. J Clin Pathol 2001; 54:890.
  7. Coppola M, Yealy DM. Relapsing polychondritis: an unusual cause of painful auricular swelling. Ann Emerg Med 1992; 21:81.
  8. Khan JH, Ahmed I. A case of relapsing polychondritis involving the tragal and the conchal bowl areas with sparing of the helix and the antihelix. J Am Acad Dermatol 1999; 41:299.
  9. Cody DT, Sones DA. Relapsing polychondritis: audiovestibular manifestations. Laryngoscope 1971; 81:1208.
  10. Clark LJ, Wakeel RA, Ormerod AD. Relapsing polychondritis--two cases with tracheal stenosis and inner ear involvement. J Laryngol Otol 1992; 106:841.
  11. Isaak BL, Liesegang TJ, Michet CJ Jr. Ocular and systemic findings in relapsing polychondritis. Ophthalmology 1986; 93:681.
  12. Sainz de la Maza M, Foster CS, Jabbur NS. Scleritis associated with systemic vasculitic diseases. Ophthalmology 1995; 102:687.
  13. Yu EN, Jurkunas U, Rubin PA, et al. Obliterative microangiopathy presenting as chronic conjunctivitis in a patient with relapsing polychondritis. Cornea 2006; 25:621.
  14. Yoo JH, Chodosh J, Dana R. Relapsing polychondritis: systemic and ocular manifestations, differential diagnosis, management, and prognosis. Semin Ophthalmol 2011; 26:261.
  15. Tucker SM, Linberg JV, Doshi HM. Relapsing polychondritis, another cause for a "salmon patch". Ann Ophthalmol 1993; 25:389.
  16. Rofail M, Lee LR, Whitehead K. Conjunctival benign reactive lymphoid hyperplasia associated with myopic scleral thinning. Clin Exp Ophthalmol 2005; 33:73.
  17. Lichauco JJ, Lauer S, Shigemitsu HH, et al. Orbital mucosa-associated lymphoid tissue (MALT)-type lymphoma in a patient with relapsing polychondritis. Arthritis Rheum 2001; 44:1713.
  18. Sheffield E, Corrin B. Fatal bronchial stenosis due to isolated relapsing chondritis. Histopathology 1992; 20:442.
  19. Tsunezuka Y, Sato H, Shimizu H. Tracheobronchial involvement in relapsing polychondritis. Respiration 2000; 67:320.
  20. Rafeq S, Trentham D, Ernst A. Pulmonary manifestations of relapsing polychondritis. Clin Chest Med 2010; 31:513.
  21. Suzuki S, Ikegami A, Hirota Y, Ikusaka M. Fever and cough without pulmonary abnormalities on CT: relapsing polychondritis restricted to the airways. Lancet 2015; 385:88.
  22. Ernst A, Rafeq S, Boiselle P, et al. Relapsing polychondritis and airway involvement. Chest 2009; 135:1024.
  23. Eng J, Sabanathan S. Airway complications in relapsing polychondritis. Ann Thorac Surg 1991; 51:686.
  24. Tillie-Leblond I, Wallaert B, Leblond D, et al. Respiratory involvement in relapsing polychondritis. Clinical, functional, endoscopic, and radiographic evaluations. Medicine (Baltimore) 1998; 77:168.
  25. Riha RL, Douglas NJ. Obstructive sleep apnoea/hypopnoea as the initial presentation of relapsing polychondritis. Int J Clin Pract 2004; 58:97.
  26. Wenig BM. Necrotizing sialometaplasia of the larynx. A report of two cases and a review of the literature. Am J Clin Pathol 1995; 103:609.
  27. O'Hanlan M, McAdam LP, Bluestone R, Pearson CM. The arthropathy of relapsing polychrondritis. Arthritis Rheum 1976; 19:191.
  28. Balsa A, Expinosa A, Cuesta M, et al. Joint symptoms in relapsing polychondritis. Clin Exp Rheumatol 1995; 13:425.
  29. Gergely P Jr, Poór G. Relapsing polychondritis. Best Pract Res Clin Rheumatol 2004; 18:723.
  30. Mathew SD, Battafarano DF, Morris MJ. Relapsing polychondritis in the Department of Defense population and review of the literature. Semin Arthritis Rheum 2012; 42:70.
  31. Lang-Lazdunski L, Hvass U, Paillole C, et al. Cardiac valve replacement in relapsing polychondritis. A review. J Heart Valve Dis 1995; 4:227.
  32. Marshall DA, Jackson R, Rae AP, Capell HA. Early aortic valve cusp rupture in relapsing polychondritis. Ann Rheum Dis 1992; 51:413.
  33. Buckley LM, Ades PA. Progressive aortic valve inflammation occurring despite apparent remission of relapsing polychondritis. Arthritis Rheum 1992; 35:812.
  34. Watanabe M, Suzuki H, Ara T, et al. Relapsing polychondritis complicated by giant cell myocarditis and myositis. Intern Med 2013; 52:1397.
  35. Chang-Miller A, Okamura M, Torres VE, et al. Renal involvement in relapsing polychondritis. Medicine (Baltimore) 1987; 66:202.
  36. Dalal BI, Wallace AC, Slinger RP. IgA nephropathy in relapsing polychondritis. Pathology 1988; 20:85.
  37. Massry GG, Chung SM, Selhorst JB. Optic neuropathy, headache, and diplopia with MRI suggestive of cerebral arteritis in relapsing polychondritis. J Neuroophthalmol 1995; 15:171.
  38. Sundaram MB, Rajput AH. Nervous system complications of relapsing polychondritis. Neurology 1983; 33:513.
  39. Erten-Lyons D, Oken B, Woltjer RL, Quinn J. Relapsing polychondritis: an uncommon cause of dementia. J Neurol Neurosurg Psychiatry 2008; 79:609.
  40. Hanslik T, Wechsler B, Piette JC, et al. Central nervous system involvement in relapsing polychondritis. Clin Exp Rheumatol 1994; 12:539.
  41. Wasserfallen JB, Schaller MD. Unusual rhombencephalitis in relapsing polychondritis. Ann Rheum Dis 1992; 51:1184.
  42. Ohta Y, Nagano I, Niiya D, et al. Nonparaneoplastic limbic encephalitis with relapsing polychondritis. J Neurol Sci 2004; 220:85.
  43. Cañas CA, Díaz-Martínez JC, Tobón GJ. Combination of hypertrophic pachymeningitis, PR3-ANCA-positive vasculitis, and relapsing polychondritis. J Rheumatol 2011; 38:966.
  44. Francès C, el Rassi R, Laporte JL, et al. Dermatologic manifestations of relapsing polychondritis. A study of 200 cases at a single center. Medicine (Baltimore) 2001; 80:173.
  45. Levesque H, Kerleau JM, Ducrotte P, et al. [Esophageal involvement in atrophic polychondritis]. Presse Med 1990; 19:1056.
  46. André MF, Piette JC, Kémény JL, et al. Aseptic abscesses: a study of 30 patients with or without inflammatory bowel disease and review of the literature. Medicine (Baltimore) 2007; 86:145.
  47. Piette JC, El-Rassi R, Amoura Z. Antinuclear antibodies in relapsing polychondritis. Ann Rheum Dis 1999; 58:656.
  48. Balsa-Criado A, Gonzalez-Hernandez T, Cuesta MV, et al. Lupus anticoagulant in relapsing polychondritis. J Rheumatol 1990; 17:1426.
  49. Foidart JM, Abe S, Martin GR, et al. Antibodies to type II collagen in relapsing polychondritis. N Engl J Med 1978; 299:1203.
  50. Ebringer R, Rook G, Swana GT, et al. Autoantibodies to cartilage and type II collagen in relapsing polychondritis and other rheumatic diseases. Ann Rheum Dis 1981; 40:473.
  51. Charrière G, Hartmann DJ, Vignon E, et al. Antibodies to types I, II, IX, and XI collagen in the serum of patients with rheumatic diseases. Arthritis Rheum 1988; 31:325.
  52. Terato K, Shimozuru Y, Katayama K, et al. Specificity of antibodies to type II collagen in rheumatoid arthritis. Arthritis Rheum 1990; 33:1493.
  53. Yang CL, Brinckmann J, Rui HF, et al. Autoantibodies to cartilage collagens in relapsing polychondritis. Arch Dermatol Res 1993; 285:245.
  54. Alsalameh S, Mollenhauer J, Scheuplein F, et al. Preferential cellular and humoral immune reactivities to native and denatured collagen types IX and XI in a patient with fatal relapsing polychondritis. J Rheumatol 1993; 20:1419.
  55. Hansson AS, Heinegård D, Piette JC, et al. The occurrence of autoantibodies to matrilin 1 reflects a tissue-specific response to cartilage of the respiratory tract in patients with relapsing polychondritis. Arthritis Rheum 2001; 44:2402.
  56. Herman JH, Dennis MV. Immunopathologic studies in relapsing polychondritis. J Clin Invest 1973; 52:549.
  57. Papo T, Piette JC, Le Thi Huong Du, et al. Antineutrophil cytoplasmic antibodies in polychondritis. Ann Rheum Dis 1993; 52:384.
  58. Handrock K, Gross WL. Relapsing polychondritis as a secondary phenomenon of primary systemic vasculitis. Ann Rheum Dis 1993; 52:895.
  59. Saxne T, Heinegård D. Involvement of nonarticular cartilage, as demonstrated by release of a cartilage-specific protein, in rheumatoid arthritis. Arthritis Rheum 1989; 32:1080.
  60. Saxne T, Heinegård D. Serum concentrations of two cartilage matrix proteins reflecting different aspects of cartilage turnover in relapsing polychondritis. Arthritis Rheum 1995; 38:294.
  61. Kraus VB, Stabler T, Le ET, et al. Urinary type II collagen neoepitope as an outcome measure for relapsing polychondritis. Arthritis Rheum 2003; 48:2942.
  62. Kempta Lekpa F, Piette JC, Bastuji-Garin S, et al. Serum cartilage oligomeric matrix protein (COMP) level is a marker of disease activity in relapsing polychondritis. Clin Exp Rheumatol 2010; 28:553.
  63. Letko E, Zafirakis P, Baltatzis S, et al. Relapsing polychondritis: a clinical review. Semin Arthritis Rheum 2002; 31:384.
  64. Zeuner M, Straub RH, Rauh G, et al. Relapsing polychondritis: clinical and immunogenetic analysis of 62 patients. J Rheumatol 1997; 24:96.
  65. Barzegar C, Vrtovsnik F, Devars JF, et al. Vasculitis with mesangial IgA deposits complicating relapsing polychondritis. Clin Exp Rheumatol 2002; 20:89.
  66. Cipriano PR, Alonso DR, Baltaxe HA, et al. Multiple aortic aneurysms in relapsing polychondritis. Am J Cardiol 1976; 37:1097.
  67. Michet CJ Jr, McKenna CH, Luthra HS, O'Fallon WM. Relapsing polychondritis. Survival and predictive role of early disease manifestations. Ann Intern Med 1986; 104:74.
  68. Maillefert JF, De Wazières B, Meignan F. Orchitis: an unusual feature in relapsing polychondritis. J Rheumatol 1994; 21:1378.
  69. Weber F, Kowald E, Schmuth M, Sepp N. Microscopic polyangiitis in a patient with relapsing polychondritis. Rheumatology (Oxford) 2001; 40:233.
  70. Firestein GS, Gruber HE, Weisman MH, et al. Mouth and genital ulcers with inflamed cartilage: MAGIC syndrome. Five patients with features of relapsing polychondritis and Behçet's disease. Am J Med 1985; 79:65.
  71. Orme RL, Nordlund JJ, Barich L, Brown T. The MAGIC syndrome (mouth and genital ulcers with inflamed cartilage). Arch Dermatol 1990; 126:940.
  72. Manganelli P, Delsante G, Bianchi G, et al. Remitting seronegative symmetrical synovitis with pitting oedema in a patient with myelodysplastic syndrome and relapsing polychondritis. Clin Rheumatol 2001; 20:132.
  73. Herrera I, Concha R, Molina EG, et al. Relapsing polychondritis, chronic hepatitis C virus infection, and mixed cryoglobulemia. Semin Arthritis Rheum 2004; 33:388.
  74. Hebbar M, Brouillard M, Wattel E, et al. Association of myelodysplastic syndrome and relapsing polychondritis: further evidence. Leukemia 1995; 9:731.
  75. Tomomatsu J, Hamano Y, Ando J, et al. Non-myeloablative allogenic BMT for myelodysplastic syndrome successfully controlled accompanying relapsing polychondritis. Bone Marrow Transplant 2012; 47:742.
  76. Yanagi T, Matsumura T, Kamekura R, et al. Relapsing polychondritis and malignant lymphoma: is polychondritis paraneoplastic? Arch Dermatol 2007; 143:89.
  77. Karaca NE, Aksu G, Yildiz B, et al. Relapsing polychondritis in a child with common variable immunodeficiency. Int J Dermatol 2009; 48:525.
  78. Gavrilova T, Capitle E. A case of relapsing polychondritis and hypogammaglobulinemia. Ann Allergy Asthma Immunol 2013; 111:147.
  79. Dolev JC, Maurer TA, Reddy SG, et al. Relapsing polychondritis in HIV-infected patients: a report of two cases. J Am Acad Dermatol 2004; 51:1023.
  80. Papo T, Wechsler B, Bletry O, et al. Pregnancy in relapsing polychondritis: twenty-five pregnancies in eleven patients. Arthritis Rheum 1997; 40:1245.