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Case Reports
. 2014 Dec 20:9:198.
doi: 10.1186/s13023-014-0198-1.

Relapsing polychondritis: clinical presentations, disease activity and outcomes

Aman Sharma  1 , Arjun Dutt Law  2 , Pradeep Bambery  3 , Vinay Sagar  4 , Ajay Wanchu  5 , Varun Dhir  6 , Rajesh Vijayvergiya  7 , Kusum Sharma  8 , Ashok Gupta  9 , Naresh K Panda  10 , Surjit Singh  11
Affiliations
Case Reports

Relapsing polychondritis: clinical presentations, disease activity and outcomes

Aman Sharma et al. Orphanet J Rare Dis. .

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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Figures

Figure 1
Figure 1
Kaplan Meier survival curve of relapsing polychondritis (n = 26).
Figure 2
Figure 2
Scatter plot of patients showing RPDAI scores.
See this image and copyright information in PMC

References

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