Relapsing polychondritis: clinical presentations, disease activity and outcomes

Sharma, Aman, Law, Arjun Dutt, Bambery, Pradeep, Sagar, Vinay, Wanchu, Ajay, Dhir, Varun, Vijayvergiya, Rajesh, Sharma, Kusum, Gupta, Ashok, Panda, Naresh K. and Singh, Surjit (2014) Relapsing polychondritis: clinical presentations, disease activity and outcomes. Orphanet Journal of Rare Diseases, 9 198: 1-6. doi:10.1186/s13023-014-0198-1

Author Sharma, Aman
Law, Arjun Dutt
Bambery, Pradeep
Sagar, Vinay
Wanchu, Ajay
Dhir, Varun
Vijayvergiya, Rajesh
Sharma, Kusum
Gupta, Ashok
Panda, Naresh K.
Singh, Surjit
Title Relapsing polychondritis: clinical presentations, disease activity and outcomes
Journal name Orphanet Journal of Rare Diseases   Check publisher's open access policy
ISSN 1750-1172
Publication date 2014-12-20
Year available 2014
Sub-type Article (original research)
DOI 10.1186/s13023-014-0198-1
Open Access Status DOI
Volume 9
Issue 198
Start page 1
End page 6
Total pages 6
Place of publication London, United Kingdom
Publisher BioMed Central
Collection year 2015
Language eng
Formatted abstract

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.


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.


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).


Apart from reduced laryngotracheal involvement, RP in India was clinically similar to recorded patterns elsewhere.
Keyword Relapsing polychondritis
Auricular chondritis
MAGIC syndrome
Myelodysplastic syndrome
Behcets disease
Q-Index Code C1
Q-Index Status Confirmed Code
Institutional Status UQ

Document type: Journal Article
Sub-type: Article (original research)
Collections: Official 2015 Collection
School of Medicine Publications
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