Relapsing polychondritis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] ,Associate Editor(s)-in-Chief: Manpreet Kaur, MD [2]

Overview

Historical Perspective

  • In 1923, Jaksch-Wartenhorst used the term polychondropathia to describe his first case about relapsing polychondritis.
  • In 1960, the term relapsing polychondritis was first coined by Pearson.

Classification

  • There is no established classification of relapsing polychondritis.

Pathophysiology

Differentiating Relapsing Polychondritis from other Diseases

Epidemiology and Demographics

  • The incidence of relapsing polychondritis is approximately 4 per 100,000 individuals in Minnesota.

Age

  • Relapsing polychondritis is more commonly observed among patients aged 40 to 60 years but it can also occur in childhood.[2][3]

Gender

  • Relapsing polychondritis affects men and women equally.

Race

  • Relapsing polychondritis usually affects Caucasian.

Risk Factors

Common risk factors in the development of relapsing polychondritis include:

Natural History, Complications and Prognosis

  • The majority of patients with relapsing polychondritis remain asymptomatic for several years.
  • Early clinical features include ear pain, polyarthritis and other non-specific symptoms such as fever, weight loss and skin rash.
  • If left untreated, [#%] of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
  • Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].
  • Prognosis is generally [excellent/good/poor], and the [1/5/10­year mortality/survival rate] of patients with [disease name] is approximately [#%].

Diagnosis

Diagnostic Criteria

Mc Adam diagnostic criteria for relapsing polychondritis[4]
Criteria Requirement
Recurrent chondritis of both auricles 3 out of 6 features

are required for definitive

diagnosis of relapsing

polychondritis

Non-erosive inflammatory polyarthritis
Nasal chondritis
Inflammation of auricular structures 
Chondritis of the respiratory tract
Cochlear and/or vestibular damage

According to modified Damiani criteria, there should be one of the following findings to diagnose relapsing polychondritis:[5]

  • There should be three of McAdam's diagnostic criteria.
  • One or more of the clinical findings of McAdam's diagnostic criteria and positive histologic confirmation.
  • Chondritis at two or more separate anatomic locations with a response to glucocorticoids or dapsone.

Symptoms

Symptoms of relapsing polychondritis may include the following:[6][7][8][9]

Physical Examination

Laboratory Findings

Imaging Findings

Relapsing polychondritis
Source: Case courtesy of Dr Charlie Chia-Tsong Hsu[17][18]

Treatment

Medical Therapy

  • The mainstay of therapy for relapsing polychondritis is medical therapy.
  • NSAIDs are used as an initial treatment of relapsing polychondritis.[19]
  • Preferred regimen: Naproxen 500 mg PO q12h or ibuprofen 800 mg PO q6h x 7 to 10 days.
  • If NSAIDs are contraindicated or resistant then following regimens are used:
    • Preferred regimen: Dapsone 50 to 100 mg PO q24h x 4 months depending on the severity of the disease.
    • Preferred regimen: Prednisone 30 to 60 mg PO q24h in divided dose.

Treatment of relapse:

Treatment of life-threatening organ damage:

  • Preferred regimen: Initial therapy is the combination of prednisone 1 mg/kg PO q24h and cyclophosphamide 2 mg/kg PO q24h.
  • Maintenance therapy depends on the renal function of a patient.
  • Patient without nephritis, Preferred regimen: Methotrexate 15mg PO once weekly.
  • Patient with nephritis, Preferred regimen: Azathioprine

Surgery

Medical therapy is the mainstay of therapy for relapsing polychondritis, however various surgical options include:

Prevention

  • There are no primary preventive measures available for [disease name].
  • Effective measures for the primary prevention of [disease name] include [measure1], [measure2], and [measure3].
  • Once diagnosed and successfully treated, patients with [disease name] are followed-up every [duration]. Follow-up testing includes [test 1], [test 2], and [test 3].

References

  1. Lang B, Rothenfusser A, Lanchbury JS, Rauh G, Breedveld FC, Urlacher A, Albert ED, Peter HH, Melchers I (May 1993). "Susceptibility to relapsing polychondritis is associated with HLA-DR4". Arthritis Rheum. 36 (5): 660–4. PMID 8489544.
  2. Knipp S, Bier H, Horneff G, Specker C, Schuster A, Schroten H, Lenard HG, Niehues T (2000). "Relapsing polychondritis in childhood--case report and short review". Rheumatol. Int. 19 (6): 231–4. PMID 11063294.
  3. Belot A, Duquesne A, Job-Deslandre C, Costedoat-Chalumeau N, Boudjemaa S, Wechsler B, Cochat P, Piette JC, Cimaz R (March 2010). "Pediatric-onset relapsing polychondritis: case series and systematic review". J. Pediatr. 156 (3): 484–9. doi:10.1016/j.jpeds.2009.09.045. PMID 19880136.
  4. McAdam LP, O'Hanlan MA, Bluestone R, Pearson CM (May 1976). "Relapsing polychondritis: prospective study of 23 patients and a review of the literature". Medicine (Baltimore). 55 (3): 193–215. PMID 775252.
  5. Damiani JM, Levine HL (June 1979). "Relapsing polychondritis--report of ten cases". Laryngoscope. 89 (6 Pt 1): 929–46. PMID 449538.
  6. Coppola M, Yealy DM (January 1992). "Relapsing polychondritis: an unusual cause of painful auricular swelling". Ann Emerg Med. 21 (1): 81–5. PMID 1539895.
  7. Kent PD, Michet CJ, Luthra HS (January 2004). "Relapsing polychondritis". Curr Opin Rheumatol. 16 (1): 56–61. PMID 14673390.
  8. O'Hanlan M, McAdam LP, Bluestone R, Pearson CM (1976). "The arthropathy of relapsing polychrondritis". Arthritis Rheum. 19 (2): 191–4. PMID 1259793.
  9. Balsa A, Expinosa A, Cuesta M, MacLeod TI, Gijón-Baños J, Maddison PJ (1995). "Joint symptoms in relapsing polychondritis". Clin. Exp. Rheumatol. 13 (4): 425–30. PMID 7586772.
  10. Foidart JM, Abe S, Martin GR, Zizic TM, Barnett EV, Lawley TJ, Katz SI (November 1978). "Antibodies to type II collagen in relapsing polychondritis". N. Engl. J. Med. 299 (22): 1203–7. doi:10.1056/NEJM197811302992202. PMID 714080.
  11. Ebringer R, Rook G, Swana GT, Bottazzo GF, Doniach D (October 1981). "Autoantibodies to cartilage and type II collagen in relapsing polychondritis and other rheumatic diseases". Ann. Rheum. Dis. 40 (5): 473–9. PMC 1000784. PMID 7030234.
  12. Papo T, Piette JC, Le Thi Huong D, Godeau P, Meyer O, Kahn MF, Bourgeois P (May 1993). "Antineutrophil cytoplasmic antibodies in polychondritis". Ann. Rheum. Dis. 52 (5): 384–5. PMC 1005055. PMID 8323388. Vancouver style error: initials (help)
  13. Passos CO, Onofre GR, Martins RC, Graff DL, Pagani EA, Sodré CT, Silva LC (July 2002). "Composition of urinary glycosaminoglycans in a patient with relapsing polychondritis". Clin. Biochem. 35 (5): 377–81. PMID 12270767.
  14. Kraus VB, Stabler T, Le ET, Saltarelli M, Allen NB (October 2003). "Urinary type II collagen neoepitope as an outcome measure for relapsing polychondritis". Arthritis Rheum. 48 (10): 2942–8. doi:10.1002/art.11281. PMID 14558101.
  15. Kempta Lekpa F, Piette JC, Bastuji-Garin S, Kraus VB, Stabler TV, Poole AR, Marini-Portugal A, Chevalier X (2010). "Serum cartilage oligomeric matrix protein (COMP) level is a marker of disease activity in relapsing polychondritis". Clin. Exp. Rheumatol. 28 (4): 553–5. PMID 20810035.
  16. Lee KS, Ernst A, Trentham DE, Lunn W, Feller-Kopman DJ, Boiselle PM (August 2006). "Relapsing polychondritis: prevalence of expiratory CT airway abnormalities". Radiology. 240 (2): 565–73. doi:10.1148/radiol.2401050562. PMID 16801364.
  17. href="https://radiopaedia.org/">Radiopaedia.org
  18. href="https://radiopaedia.org/cases/31793">rID: 31793
  19. Yoo JH, Chodosh J, Dana R (2011). "Relapsing polychondritis: systemic and ocular manifestations, differential diagnosis, management, and prognosis". Semin Ophthalmol. 26 (4–5): 261–9. doi:10.3109/08820538.2011.588653. PMID 21958172.

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