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Orphanet Journal of Rare Diseases
Published in: Orphanet Journal of Rare Diseases 1/2014

Open Access 01-12-2014 | Research

Relapsing polychondritis: clinical presentations, disease activity and outcomes

Authors: Aman Sharma, Arjun Dutt Law, Pradeep Bambery, Vinay Sagar, Ajay Wanchu, Varun Dhir, Rajesh Vijayvergiya, Kusum Sharma, Ashok Gupta, Naresh K Panda, Surjit Singh

Published in: Orphanet Journal of Rare Diseases | Issue 1/2014

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Abstract

Background

Relapsing polychondritis is a rare disease characterised by inflammation of cartilaginous and proteoglycan rich structures. As there are only a few published single centre case series from all across the world, we describe our experience with 26 patients at a tertiary centre in north India.

Methods

A retrospective study with all patients meeting Damiani and Levine’s modification of McAdam’s diagnostic criteria. Clinical details, investigations, disease activity assessment [(Relapsing Polychondritis Disease Activity Index (RPDAI)], treatment and outcomes were recorded.

Results

Ten men and sixteen women (median age 45 years) met the diagnostic criteria. Auricular chondritis (96%), arthritis (54%), hearing impairment (42%), ocular (42%), dermal (26%), cardiovascular (11%) and laryngotracheal involvement (11%) characterized the clinical presentations. The median RPDAI was 31 (range 9-66). Two patients died during observation. Overall survival was 92.3% (median survival 13.5 years).

Conclusions

Apart from reduced laryngotracheal involvement, RP in India was clinically similar to recorded patterns elsewhere.
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Literature
1.
McAdam LP, O'Hanlan MA, Bluestone R, Pearson CM: Relapsing polychondritis: prospective study of 23 patients and a review of the literature. Medicine (Baltimore). 1976, 55: 193-215. 10.1097/00005792-197605000-00001.CrossRef
2.
Michet CJ, McKenna CH, Luthra HS, O'Fallon WM: Relapsing polychondritis. Survival and predictive role of early disease manifestations. Ann Intern Med. 1986, 104: 74-78. 10.7326/0003-4819-104-1-74.CrossRefPubMed
3.
Trentham DE, Le CH: Relapsing polychondritis. Ann Intern Med. 1998, 129: 114-122. 10.7326/0003-4819-129-2-199807150-00011.CrossRefPubMed
4.
Kong KO, Vasoo S, Tay NS, Chng HH: Relapsing polychondritis–an Oriental case series. Singapore Med J. 2003, 44: 197-200.PubMed
5.
Sharma A, Bambery P, Wanchu A, Sharma YP, Panda NK, Gupta A, Singh S: Relapsing polychondritis in North India: a report of 10 patients. Scand J Rheumatol. 2007, 36: 462-465. 10.1080/03009740701406502.CrossRefPubMed
6.
Ananthakrishna R, Goel R, Padhan P, Mathew J, Danda D: Relapsing polychondritis--case series from South India. Clin Rheumatol. 2009, 28: S7-S10. 10.1007/s10067-008-1016-8.CrossRefPubMed
7.
Mathew SD, Battafarano DF, Morris MJ: Relapsing polychondritis in the Department of Defense population and review of the literature. Semin Arthritis Rheum. 2012, 42: 70-83. 10.1016/j.semarthrit.2011.12.007.CrossRefPubMed
8.
Damiani JM, Levine HL: Relapsing polychondritis–report of ten cases. Laryngoscope. 1979, 89: 929-946. 10.1288/00005537-197906000-00009.CrossRefPubMed
9.
Arnaud L, Devilliers H, Peng SL, Mathian A, Costedoat-Chalumeau N, Buckner J, Dagna L, Michet C, Sharma A, Cervera R, Haroche J, Papo T, D'Cruz D, Arlet P, Zwerina J, Belot A, Suzuki N, Harle JR, Moots R, Jayne D, Hachulla E, Marie I, Tanaka T, Lebovics R, Scott D, Kucharz EJ, Birchall M, Kong KO, Gorochov G, Amoura Z: The Relapsing Polychondritis Disease Activity Index: development of a disease activity score for relapsing polychondritis. Autoimmun Rev. 2012, 12: 204-209. 10.1016/j.autrev.2012.06.005.CrossRefPubMed
10.
Arnaud L, Mathian A, Haroche J, Gorochov G, Amoura Z: Pathogenesis of relapsing polychondritis: a 2013 update. Autoimmun Rev. 2014, 13: 90-95. 10.1016/j.autrev.2013.07.005.CrossRefPubMed
11.
Takagi D, Iwabuchi K, Iwabuchi C, Nakamaru Y, Maguchi S, Ohwatari R, Furuta Y, Fukuda S, Joyce S, Onoe K: Immunoregulatory defects of V alpha 24 V+ beta 11+ NKT cells in development of Wegener’s granulomatosis and relapsing polychondritis. Clin Exp Immunol. 2004, 136: 591-600. 10.1111/j.1365-2249.2004.02471.x.CrossRefPubMedPubMedCentral
12.
Zeuner M, Straub RH, Rauh G, Albert ED, Scholmerich J, Lang B: Relapsing polychondritis: clinical and immunogenetic analysis of 62 patients. J Rheumatol. 1997, 24: 96-101.PubMed
13.
Knipp S, Bier H, Horneff G, Specker C, Schuster A, Schroten H, Lenard HG, Niehues T: Relapsing polychondritis in childhood–case report and short review. Rheumatol Int. 2000, 19: 231-234. 10.1007/s002960000055.CrossRefPubMed
14.
Fonseca AR, de Oliveira SK, Rodrigues MC, Aymore IL, Domingues RC, Sztajnbok FR: Relapsing polychondritis in childhood: three case reports, comparison with adulthood disease and literature review. Rheumatol Int. 2013, 33: 1873-1878. 10.1007/s00296-011-2336-6.CrossRefPubMed
15.
Belot A, Duquesne A, Job-Deslandre C, Costedoat-Chalumeau N, Boudjemaa S, Wechsler B, Cochat P, Piette JC, Cimaz R: Pediatric-onset relapsing polychondritis: case series and systematic review. J Pediatr. 2010, 156: 484-489. 10.1016/j.jpeds.2009.09.045.CrossRefPubMed
16.
Isaak BL, Liesegang TJ, Michet CJ: Ocular and systemic findings in relapsing polychondritis. Ophthalmology. 1986, 93: 681-689. 10.1016/S0161-6420(86)33695-9.CrossRefPubMed
17.
Michelson JB: Melting corneas with collapsing nose. Surv Ophthalmol. 1984, 29: 148-154. 10.1016/0039-6257(84)90172-3.CrossRefPubMed
18.
Chang SJ, Lu CC, Chung YM, Lee SS, Chou CT, Huang DF: Laryngotracheal involvement as the initial manifestation of relapsing polychondritis. J Chin Med Assoc. 2005, 68: 279-282. 10.1016/S1726-4901(09)70151-0.CrossRefPubMed
19.
Firestein GS, Gruber HE, Weisman MH, Zvaifler NJ, Barber J, O'Duffy JD: Mouth and genital ulcers with inflamed cartilage: MAGIC syndrome. Five patients with features of relapsing polychondritis and Behcet’s disease. Am J Med. 1985, 79: 65-72. 10.1016/0002-9343(85)90547-9.CrossRefPubMed
20.
Chang-Miller A, Okamura M, Torres VE, Michet CJ, Wagoner RD, Donadio JV, Offord KP, Holley KE: Renal involvement in relapsing polychondritis. Medicine (Baltimore). 1987, 66: 202-217. 10.1097/00005792-198705000-00004.CrossRef
21.
Sharma A, Kumar S, Goyal P, Law AD, Lal A, Singh S: Steroid responsive parenchymal lung involvement and acute renal failure in a patient with relapsing polychondritis. J Postgrad Med Edu Res. 2012, 46: 198-201. 10.5005/jp-journals-10028-1045.CrossRef
22.
Hebbar M, Brouillard M, Wattel E, Decoulx M, Hatron PY, Devulder B, Fenaux P: Association of myelodysplastic syndrome and relapsing polychondritis: further evidence. Leukemia. 1995, 9: 731-733.PubMed
23.
Salahuddin N, Libman BS, Lunde JH, Kay J, Cooper SM: The association of relapsing polychondritis and myelodysplastic syndrome: report of three cases. J Clin Rheumatol. 2000, 6: 146-149. 10.1097/00124743-200006000-00006.CrossRefPubMed
24.
Frances C, el Rassi R, Laporte JL, Rybojad M, Papo T, Piette JC: Dermatologic manifestations of relapsing polychondritis. A study of 200 cases at a single center. Medicine (Baltimore). 2001, 80: 173-179. 10.1097/00005792-200105000-00003.CrossRef
25.
Washio K, Oka M, Ohno K, Shimizu H, Kawano S, Kunisada M, Nishigori C: Case of recurrent Sweet’s syndrome in a patient with relapsing polychondritis and myelodysplastic syndrome. J Dermatol. 2012, 39: 731-733. 10.1111/j.1346-8138.2011.01403.x.CrossRefPubMed
26.
Yamashita H, Takahashi H, Kubota K, Ueda Y, Ozaki T, Yorifuji H, Bannai E, Minamimoto R, Morooka M, Miyata Y, Okasaki M, Takahashi Y, Kaneko H, Kano T, Mimori A: 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: 1482-1490. 10.1093/rheumatology/keu147.CrossRef
27.
Sharma A, Mittal T, Kumar S, Law AD, Wanchu A, Mahajan R, Bambery P, Singh S: Successful treatment of aortic root dilatation in a patient with relapsing polychondritis. Clin Rheumatol. 2013, 32: S59-S61. 10.1007/s10067-010-1450-2.CrossRefPubMed
28.
de Barros AP, Nakamura NA, Santana Tde F, Motta JQ, Bianchi WA: Infliximab in relapsing polychondritis. Rev Bras Reumatol. 2010, 50: 211-216. 10.1590/S0482-50042010000200010.CrossRefPubMed
29.
Subrahmanyam P, Balakrishnan C, Dasgupta B: Sustained response to etanercept after failing infliximab, in a patient with relapsing polychondritis with tracheomalacia. Scand J Rheumatol. 2008, 37: 239-240. 10.1080/03009740701747160.CrossRefPubMed
30.
Navarrete VM, Lebron CV, de Miera FJ, Candau CP, Sarabia FN: Sustained remission of pediatric relapsing polychondritis with adalimumab. J Clin Rheumatol. 2014, 20: 45-46. 10.1097/RHU.0000000000000063.CrossRefPubMed
31.
Peng SL, Rodriguez D: Abatacept in relapsing polychondritis. Ann Rheum Dis. 2013, 72: 1427-1429. 10.1136/annrheumdis-2013-203319.CrossRefPubMed
32.
Wendling D, Godfrin-Valnet M, Prati C: Treatment of relapsing polychondritis with tocilizumab. J Rheumatol. 2013, 40: 1232-10.3899/jrheum.130371.CrossRefPubMed
33.
Daikeler T, Kotter I, Bocelli Tyndall C, Apperley J, Attarbaschi A, Guardiola P, Gratwohl A, Jantunen E, Marmont A, Porretto F, Musso M, Maurer B, Rinaldi N, Saccardi R, Tyndall A: Haematopoietic stem cell transplantation for vasculitis including Behcet’s disease and polychondritis: a retrospective analysis of patients recorded in the European Bone Marrow Transplantation and European League against Rheumatism databases and a review of the literature. Ann Rheum Dis. 2007, 66: 202-207. 10.1136/ard.2006.056630.CrossRefPubMedPubMedCentral
Metadata
Title
Relapsing polychondritis: clinical presentations, disease activity and outcomes
Authors
Aman Sharma
Arjun Dutt Law
Pradeep Bambery
Vinay Sagar
Ajay Wanchu
Varun Dhir
Rajesh Vijayvergiya
Kusum Sharma
Ashok Gupta
Naresh K Panda
Surjit Singh
Publication date
01-12-2014
Publisher
BioMed Central
Published in
Orphanet Journal of Rare Diseases / Issue 1/2014
Electronic ISSN: 1750-1172
DOI
https://doi.org/10.1186/s13023-014-0198-1

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