Relapsing Polychondritis: An Updated Review

Overview

Journal Biomedicines

Specialty Biomedical Engineering

Date 2018 Aug 4

PMID 30072598

Citations 38

Authors

Francesco Borgia

Roberta Giuffrida

Fabrizio Guarneri

Serafinella P Cannavo

Affiliations

Soon will be listed here.

Abstract

Relapsing polychondritis is an immune-mediated systemic disease characterized by recurrent episodes of inflammation of cartilaginous and proteoglycan-rich tissues, resulting in progressive anatomical deformation and functional impairment of the involved structures. Auricular and nasal chondritis and/or polyarthritis represent the most common clinical features, but potentially all types of cartilage may be involved. Because of the pleomorphic nature of the disease, with non-specific symptoms at the onset, the diagnosis of relapsing polychondritis is often delayed. In this review article we provide a comprehensive look into clinical presentation, laboratory and instrumental investigations, diagnostic criteria, and therapeutic options.

Citing Articles

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References

Smylie A, Malhotra N, Brassard A . Relapsing Polychondritis: A Review and Guide for the Dermatologist. Am J Clin Dermatol. 2016; 18(1):77-86. DOI: 10.1007/s40257-016-0226-0. View

Kempta Lekpa F, Piette J, Bastuji-Garin S, Kraus V, Stabler T, Poole A . Serum cartilage oligomeric matrix protein (COMP) level is a marker of disease activity in relapsing polychondritis. Clin Exp Rheumatol. 2010; 28(4):553-5. View

Firestein G, Gruber H, Weisman M, Zvaifler N, Barber J, ODuffy J . 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(1):65-72. DOI: 10.1016/0002-9343(85)90547-9. View

Martin J, Roenigk H, Lynch W, TINGWALD F . Relapsing polychondritis treated with dapsone. Arch Dermatol. 1976; 112(9):1272-4. View

Jawad A, Burrel M, Lim K, SCOTT D . Erosive arthritis in relapsing polychondritis. Postgrad Med J. 1990; 66(779):768-70. PMC: 2426865. DOI: 10.1136/pgmj.66.779.768. View

Kashihara K, Kawada S, Takahashi Y . Autoantibodies to glutamate receptor GluRepsilon2 in a patient with limbic encephalitis associated with relapsing polychondritis. J Neurol Sci. 2009; 287(1-2):275-7. DOI: 10.1016/j.jns.2009.08.004. View

Sharma A, Law A, Bambery P, Sagar V, Wanchu A, Dhir V . Relapsing polychondritis: clinical presentations, disease activity and outcomes. Orphanet J Rare Dis. 2014; 9:198. PMC: 4302515. DOI: 10.1186/s13023-014-0198-1. View

Mark K, Franks Jr A . Colchicine and indomethacin for the treatment of relapsing polychondritis. J Am Acad Dermatol. 2002; 46(2 Suppl Case Reports):S22-4. DOI: 10.1067/mjd.2002.105477. View

Barzegar C, Vrtovsnik F, Devars J, Mignon F, Pradalier A . Vasculitis with mesangial IgA deposits complicating relapsing polychondritis. Clin Exp Rheumatol. 2002; 20(1):89-91. View

10.

Moulis G, Sailler L, Astudillo L, Arlet P . Abatacept for relapsing polychondritis. Rheumatology (Oxford). 2010; 49(5):1019. DOI: 10.1093/rheumatology/kep436. View

11.

Wendling D, Govindaraju S, Prati C, Toussirot E, Bertolini E . Efficacy of anakinra in a patient with refractory relapsing polychondritis. Joint Bone Spine. 2008; 75(5):622-4. DOI: 10.1016/j.jbspin.2008.02.011. View

12.

Cantarini L, Vitale A, Brizi M, Caso F, Frediani B, Punzi L . Diagnosis and classification of relapsing polychondritis. J Autoimmun. 2014; 48-49:53-9. DOI: 10.1016/j.jaut.2014.01.026. View

13.

Buckner J, Van Landeghen M, Kwok W, Tsarknaridis L . Identification of type II collagen peptide 261-273-specific T cell clones in a patient with relapsing polychondritis. Arthritis Rheum. 2002; 46(1):238-44. DOI: 10.1002/1529-0131(200201)46:1<238::AID-ART10030>3.0.CO;2-M. View

14.

Zeuner M, Straub R, Rauh G, Albert E, Scholmerich J, Lang B . Relapsing polychondritis: clinical and immunogenetic analysis of 62 patients. J Rheumatol. 1997; 24(1):96-101. View

15.

Taneja V, Griffiths M, Behrens M, Luthra H, David C . Auricular chondritis in NOD.DQ8.Abetao (Ag7-/-) transgenic mice resembles human relapsing polychondritis. J Clin Invest. 2003; 112(12):1843-50. PMC: 296991. DOI: 10.1172/JCI17450. View

16.

Kingdon J, Roscamp J, Sangle S, DCruz D . Relapsing polychondritis: a clinical review for rheumatologists. Rheumatology (Oxford). 2017; 57(9):1525-1532. DOI: 10.1093/rheumatology/kex406. View

17.

Hebbar M, Brouillard M, Wattel E, Decoulx M, Hatron P, Devulder B . Association of myelodysplastic syndrome and relapsing polychondritis: further evidence. Leukemia. 1995; 9(4):731-3. View

18.

Hull R, Morgan S . The nervous system and relapsing polychondritis. Neurology. 1984; 34(4):557. DOI: 10.1212/wnl.34.4.557. View

19.

Yamashita H, Takahashi H, Kubota K, Ueda Y, Ozaki T, Yorifuji H . Utility of fluorodeoxyglucose positron emission tomography/computed tomography for early diagnosis and evaluation of disease activity of relapsing polychondritis: a case series and literature review. Rheumatology (Oxford). 2014; 53(8):1482-90. DOI: 10.1093/rheumatology/keu147. View

20.

Terrier B, Aouba A, Bienvenu B, Bloch-Queyrat C, Delair E, Mallet J . Complete remission in refractory relapsing polychondritis with intravenous immunoglobulins. Clin Exp Rheumatol. 2008; 26(1):136-8. View